Patients who were stage 1 or stage 2 who had loss of heterozygosity of 1 and 16q had about a 10% less overall survival than patients who didn't have it. For stage 3 and stage 4, it was significantly more. It was about 18%.
Wilms tumors tend to push things out of the way rather than growing into it, whereas a neuroblastoma, you look for things that would tend to grow out and around structures like blood vessels.
If you feel that you would have to do a major liver or bowel resection at the same time, that is a situation in which we would not recommend doing a primary nephrectomy.
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 24 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 24 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 24 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 24 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 24 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 24 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 24 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 24 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 24 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 24 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 24 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 24 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 24 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 24 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 24 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 24 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 24 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 24 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 24 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 24 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 24 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 24 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 24 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 24 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 24 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 24 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 24 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 24 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 24 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 24 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 24 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 24 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 24 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 24 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 24 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 24 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 24 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 24 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 24 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 24 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 24 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 24 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 24 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 24 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 24 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 24 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 24 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 24 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 24 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 24 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 24 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 24 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 24 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 24 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Peter's statements about Nephroblastoma170 statements
clinicalWilms tumors characteristically show a 'claw sign' on imaging where normal kidney is displaced into a horseshoe pattern, appearing to grab the mass coming out of it.↗
▶Ep 1 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing around them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 1 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 1 · 6:23
guidelineIn North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of children with renal tumors.↗
▶Ep 1 · 7:01
guidelinePreoperative chemotherapy is recommended if the tumor compromises the child's respiratory status, making them a poor operative candidate.↗
▶Ep 1 · 7:33
guidelineIf tumor extends into the inferior vena cava beyond the intrahepatic level (behind the liver or up to the atrium), preoperative chemotherapy is recommended.↗
▶Ep 1 · 7:57
guidelineMassive tumors that would require resection of large parts of liver or bowel should receive preoperative chemotherapy because the majority will respond.↗
▶Ep 1 · 8:21
guidelineIf the child has only one functioning kidney, preoperative chemotherapy is recommended to avoid nephrectomy.↗
▶Ep 1 · 8:33
guidelineChildren with bilateral renal tumors, Wilms tumor predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann), or multicentric tumors should not undergo primary nephrectomy.↗
▶Ep 1 · 10:38
clinicalComplication rates are higher if liver or bowel must be resected at the same time as the kidney.↗
▶Ep 1 · 11:18
guidelineIf gross tumor is left behind or the tumor is only biopsied, it is treated as a stage 3 abdominal tumor requiring 3-drug chemotherapy plus flank radiation.↗
▶Ep 1 · 14:19
quoteIf you feel that you would have to do a major liver or bowel resection at the same time, that is a situation in which we would not recommend doing a primary nephrectomy.↗
▶Ep 1 · 19:44
guidelineIn the COG staging system, treatment is determined by both local abdominal stage and disease stage.↗
▶Ep 1 · 20:01
guidelineStage 1 tumor is limited to the kidney, completely resected, with no capsular invasion, no rupture or biopsy prior to removal, no renal sinus vessel involvement, negative margins, and negative regional lymph nodes.↗
▶Ep 1 · 20:28
guidelineStage 2 tumor is completely resected with negative margins but extends beyond the kidney through capsular penetration, renal sinus soft tissue invasion, or blood vessel involvement outside the primary kidney.↗
▶Ep 1 · 21:00
guidelineStage 3 includes tumors that are biopsied with gross residual, have positive lymph nodes, penetrate the peritoneal surface with implants, have positive margins, have microscopic residual from intraoperative spill, cannot be completely resected, or must be removed in pieces.↗
▶Ep 1 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain.↗
▶Ep 1 · 22:06
guidelineStage 5 is bilateral renal tumor involvement.↗
▶Ep 1 · 23:08
guidelinePatients with stage 1 or 2 abdominal disease without lung metastases receive only 2-drug chemotherapy (vincristine and dactinomycin) for shorter duration with lower toxicity and significantly lower risk of late effects.↗
▶Ep 1 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy problems in females, hypertension, and cardiovascular disease, primarily caused by radiation and doxorubicin.↗
▶Ep 1 · 23:37
quoteThe two main factors that contribute to late effects are radiation and doxorubicin.↗
▶Ep 1 · 24:06
guidelineIf a child has stage 1 or 2 abdominal disease, they do not require abdominal radiation regardless of lung metastases status.↗
▶Ep 1 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer.↗
▶Ep 1 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 1 · 26:20
clinicalPulmonary radiation causes pneumonitis and long-term restrictive lung disease.↗
▶Ep 1 · 26:24
clinicalA recent COG study showed that approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks of chemotherapy do not need pulmonary radiation, with 80-85% remaining relapse-free.↗
▶Ep 1 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 1 · 30:31
guidelineIn the SIOP protocols, all patients start with chemotherapy without biopsy in most cases, using higher doses of two drugs, with evaluation at 4 and 8 weeks before proceeding to resection.↗
▶Ep 1 · 31:06
guidelineSIOP uses post-chemotherapy, post-nephrectomy classification into low risk, intermediate risk, and high risk based on percentage of blastemal components and presence of anaplasia.↗
▶Ep 1 · 35:11
clinicalSome children with Wilms tumor develop acquired von Willebrand disease; in the majority of cases it is meaningless, but a few case series report significant bleeding during surgery until the tumor is removed.↗
▶Ep 1 · 41:24
guidelinePositive surgical margins or tumor rupture (intraoperative or microscopic) automatically makes the patient stage 3.↗
▶Ep 1 · 42:31
guidelineTaking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 1 · 43:32
clinicalVery low-risk patients (stage I, <550g, <2 years, favorable histology) can be treated with surgery alone; 90-95% are cured without chemotherapy, and those who relapse have 100% survival with delayed chemotherapy.↗
▶Ep 1 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 1 · 46:38
guidelineIf IVC tumor thrombus extends into the renal vein but is not adherent and comes out in one piece, it is considered stage 2.↗
▶Ep 1 · 47:43
clinicalMajor complication rates (including mortality, blood transfusions, ICU stay) increase significantly when tumor extends beyond the infrahepatic IVC to the hepatic veins or atrium, with 26-30% major morbidity in primary resection cases.↗
▶Ep 1 · 51:28
quoteI've read 6000 operative notes. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 1 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and is associated with significantly worse outcomes regardless of stage.↗
▶Ep 1 · 55:23
epidemiologicalStage 1-2 patients with loss of heterozygosity at 1p and 16q have approximately 10% lower overall survival than those without these genetic changes.↗
▶Ep 1 · 55:45
clinicalStage 3-4 patients with loss of heterozygosity at 1p and 16q have approximately 18% lower overall survival and receive 5-drug regimen M chemotherapy.↗
▶Ep 1 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 1 · 58:51
epidemiologicalEvent-free survival for unilateral Wilms tumor is approximately 88% with overall survival of 95%.↗
▶Ep 1 · 58:51
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% with overall survival of only 80%.↗
▶Ep 1 · 59:37
clinicalThe maximum response of most children with Wilms tumor to chemotherapy occurs by 12 weeks.↗
▶Ep 1 · 1:00:31
clinicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor.↗
▶Ep 1 · 1:01:02
clinicalOpen biopsy to determine favorable vs. unfavorable histology in Wilms tumor is not very accurate initially.↗
▶Ep 1 · 1:01:12
epidemiologicalIn a recent COG bilateral Wilms study of 250 patients, only one patient who met enrollment criteria turned out to have rhabdoid tumor instead of Wilms tumor.↗
▶Ep 1 · 1:02:14
epidemiologicalDiscordant pathology occurs in up to 20% of bilateral Wilms tumor patients when both kidneys are biopsied.↗
Wilms Tumor: Audio Chapter
▶Ep 2 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 2 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 2 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 2 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 2 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 2 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 2 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 2 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 2 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 2 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 2 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 2 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 2 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 2 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 2 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 2 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 2 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 2 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 2 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 2 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 2 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 2 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 2 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 2 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 2 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 2 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 2 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 2 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 2 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 2 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 2 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 2 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 2 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 2 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 2 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 2 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 2 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 2 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 2 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 2 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 2 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 2 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 2 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 2 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 2 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 2 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 2 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 2 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 2 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 2 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 2 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 2 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 2 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 2 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 2 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Wilms Tumor
▶Ep 3 · 4:16
quoteWilms tumors tend to push things out of the way rather than growing into it, whereas a neuroblastoma, you look for things that would tend to grow out and around structures like blood vessels.↗
▶Ep 3 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 3 · 6:23
guidelineIn North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of renal tumors in children.↗
▶Ep 3 · 7:33
guidelinePreoperative chemotherapy is recommended when tumor extends into the IVC beyond the infrahepatic level, as complication rates are higher with primary surgery in these cases.↗
▶Ep 3 · 7:57
guidelinePreoperative chemotherapy is indicated if the tumor is so large that major liver or bowel resection would be required, because the majority will respond to chemotherapy and complication rates are higher when these organs must be resected.↗
▶Ep 3 · 8:33
guidelineChildren with bilateral Wilms tumors, solitary functioning kidney, or genetic predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann) should receive preoperative chemotherapy rather than primary nephrectomy.↗
▶Ep 3 · 15:55
guidelineIn situations requiring preoperative chemotherapy, biopsy should be performed to determine histology, as chemotherapy differs for favorable vs unfavorable histology and for different tumor types (Wilms, rhabdoid, clear cell sarcoma).↗
▶Ep 3 · 17:19
guidelineFor biopsy, either open biopsy or large core sampling with at least 10 cores is recommended; accuracy increases between 10 and 20 cores. Fine needle aspiration or simple true-cut biopsy is insufficient because anaplasia cannot be diagnosed from these samples.↗
▶Ep 3 · 17:46
guidelineA central venous catheter or port should be placed at the time of biopsy to avoid multiple anesthetics; a single lumen is preferred due to lower infection risk.↗
▶Ep 3 · 19:44
guidelineCOG staging distinguishes abdominal stage from disease stage. Stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels not involved, negative margins and nodes.↗
▶Ep 3 · 20:28
guidelineStage 2 is complete resection with tumor extending beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 3 · 21:00
guidelineStage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from spillage, incomplete resection, piecemeal removal, or dividing the renal vein with tumor present.↗
▶Ep 3 · 21:35
quoteIf you just biopsy and got chemotherapy, that is considered stage 3.↗
▶Ep 3 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain. Stage 5 is bilateral renal involvement.↗
▶Ep 3 · 22:20
guidelineA patient can have stage 1 or 2 abdominal disease with lung metastases (stage 4 disease stage). Such patients receive chemotherapy for metastatic disease but do not require abdominal radiation if abdominal stage is 1 or 2.↗
▶Ep 3 · 23:08
guidelineStage 1 and 2 abdominal disease without metastases receives two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks, which has lower toxicity and significantly lower risk of late effects than three-drug therapy with doxorubicin or radiation.↗
▶Ep 3 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy complications in females, hypertension, and cardiovascular disease. The two main contributing factors are radiation and doxorubicin.↗
▶Ep 3 · 23:54
quoteThe two main factors that contribute to late effects are radiation and doxorubicin.↗
▶Ep 3 · 24:15
guidelineIf a patient has a lung lesion but the abdominal tumor is resectable, primary nephrectomy should still be performed. Biopsying the abdominal tumor would automatically create stage 3 disease requiring abdominal radiation, whereas removing it with negative nodes avoids abdominal radiation.↗
▶Ep 3 · 24:52
quoteIf you had gone ahead and removed the tumor and the lymph nodes were negative, you would not necessarily have gotten abdominal radiation.↗
▶Ep 3 · 26:02
clinicalA recent COG trial showed that 40% of patients with pulmonary metastases who achieve complete response by 6 weeks of chemotherapy do not need pulmonary radiation, with 80-85% remaining disease-free. Those who relapse can be salvaged with radiation.↗
▶Ep 3 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, making radiation avoidance in responders a significant benefit.↗
▶Ep 3 · 26:08
quote15% of the girls who get pulmonary radiation from Wilm's tumor end up getting breast cancer, which is significant.↗
▶Ep 3 · 27:54
guidelineFor patients with lung lesions and resectable abdominal tumor, the approach is to perform nephrectomy and start chemotherapy based on kidney pathology without biopsying the lung lesion initially. At 6 weeks, if a single lesion remains or lesions don't respond, thoracoscopic biopsy may be performed, as 50-60% of residual lesions are not cancer.↗
▶Ep 3 · 28:29
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 3 · 29:50
guidelineAll patients with lung lesions presumed to be metastatic disease start with three-drug chemotherapy.↗
▶Ep 3 · 30:15
guidelineThe SIOP protocols used in Western Europe give preoperative chemotherapy to all patients without biopsy, using higher doses of vincristine and dactinomycin than COG protocols, with response evaluation at 4 and 8 weeks before proceeding to nephrectomy.↗
▶Ep 3 · 31:06
guidelineSIOP uses post-chemotherapy, post-nephrectomy classification into low, intermediate, and high risk. High-risk patients receive abdominal radiation regardless of lymph node status; intermediate-risk patients receive radiation if lymph node positive.↗
▶Ep 3 · 31:42
clinicalIn SIOP protocols, predominantly blastemal component after preoperative chemotherapy is a negative prognostic factor, but blastemal percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 3 · 32:45
clinicalThere is no outcome difference between patients who undergo empiric biopsy versus those who have intraoperative spillage—both are stage 3. The main prognostic factor in stage 3 is lymph node status.↗
▶Ep 3 · 33:54
clinicalOutcomes for stage 1 and 2 patients are essentially identical between COG and SIOP approaches.↗
▶Ep 3 · 33:54
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 3 · 34:46
guidelinePreoperative assessment should include hemoglobin (some tumors rupture internally causing anemia), PT/PTT (acquired von Willebrand disease can occur, rarely causing significant bleeding), and electrolytes.↗
▶Ep 3 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 3 · 37:24
guidelineSurgical approach uses transverse or modified subcostal incision, not median or retroperitoneal incisions. The tumor is mobilized completely before identifying vessels, as attempting early hilar dissection on large tumors can be dangerous.↗
▶Ep 3 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized, it's even back in Gross's book that if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 3 · 39:49
guidelineLymph node sampling should aim for at least 5-6 nodes from the renal hilum and para-aortic/paracaval regions, though evidence for a specific number is limited.↗
▶Ep 3 · 40:19
opinionTaking the renal artery before the vein is preferred by principle but not always possible with large tumors. The order of vessel division does not significantly impact outcomes.↗
▶Ep 3 · 41:24
guidelinePositive surgical margins automatically make the tumor stage 3. Microscopic rupture through the capsule that is only visible on pathology also creates stage 3 disease.↗
▶Ep 3 · 41:37
quoteSometimes the tumor can extend and kind of microscopically rupture, and you can't tell that until they look under the microscope and there's nothing you can do about it.↗
▶Ep 3 · 42:31
clinicalTaking portions of adherent diaphragm or liver to avoid tumor violation does not upstage the tumor if true invasion has not occurred—these tumors often cause intense inflammatory reaction that mimics invasion.↗
▶Ep 3 · 43:16
quoteSometimes these tumors cause an intense inflammation reaction that makes it seem like it's invaded, but it actually hasn't, and other times it has invaded and you deal with that.↗
▶Ep 3 · 43:32
clinicalVery low-risk patients (stage 1, favorable histology, tumor <550g, age <2 years) can be treated with surgery alone. This group has 90-95% cure rate without chemotherapy, and the 5-10% who relapse have 100% salvage rate.↗
▶Ep 3 · 44:08
quoteThese patients that no matter what you did, whether you treated them with surgery, whether you added 1 drug of chemotherapy, 2 drugs of chemotherapy, 3 drugs, radiation, they had excellent overall survival.↗
▶Ep 3 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor.↗
▶Ep 3 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if properly managed. Tumors extending into the renal vein that come out in one piece are considered stage 2.↗
▶Ep 3 · 46:49
guidelineFor IVC thrombus below the infrahepatic level, intraoperative ultrasound should be used to assess extent. The ideal technique is to mobilize the kidney, ligate the renal artery and accessory veins, make a small nick in the renal vein, and slide the thrombus out intact.↗
▶Ep 3 · 47:43
clinicalTumors extending above the infrahepatic IVC have significantly higher complication rates (26-30% major morbidity) with primary surgery and should receive preoperative chemotherapy.↗
▶Ep 3 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up.↗
▶Ep 3 · 51:28
quoteTodd, I've read 6000 operative notes. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 3 · 51:28
clinicalAfter reviewing 6000 operative notes, cases of tumor thrombus completely filling the IVC but stopping at the infrahepatic level are extremely rare—when the IVC is truly filled, the thrombus typically extends higher.↗
▶Ep 3 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastemal, stromal, and epithelial (triphasic). Tumors with only two components are also considered favorable. In COG protocols, the amount of blastema has never correlated with outcome.↗
▶Ep 3 · 53:13
quoteIn the children's oncology group protocols, the amount of blastoma has never correlated with outcome.↗
▶Ep 3 · 53:45
guidelineStage 1 and 2 favorable histology without metastases receives two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks. Stage 3 adds doxorubicin (DD4A regimen) for 25 weeks.↗
▶Ep 3 · 54:25
clinicalLoss of heterozygosity (LOH) testing is performed on all favorable histology tumors. Patients with both 1p and 16q LOH (5-7% of cases) have significantly worse outcomes: 10% lower survival for stage 1-2, 18% lower for stage 3-4.↗
▶Ep 3 · 55:23
guidelinePatients with both 1p and 16q LOH receive intensified treatment: stage 1-2 patients add doxorubicin (three-drug therapy), and stage 3-4 patients receive regimen M (five-drug chemotherapy).↗
▶Ep 3 · 55:23
quotePatients who were stage 1 or stage 2 who had loss of heterozygosity of 1 and 16q had about a 10% less overall survival than patients who didn't have it. For stage 3 and stage 4, it was significantly more. It was about 18%.↗
▶Ep 3 · 55:58
guidelineUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on the number of high-power fields showing anaplasia. Treatment ranges from three-drug to five-drug chemotherapy plus or minus radiation depending on extent.↗
▶Ep 3 · 56:51
quoteRenal cell carcinoma, which we don't have really good therapy for. Well, we actually don't have any good therapy for, particularly for metastatic disease.↗
▶Ep 3 · 56:51
clinicalOther renal tumors include renal cell carcinoma (second most common, no good therapy for metastatic disease), clear cell sarcoma (reasonable treatment for low stages), and rhabdoid tumor (poor outcomes except stage 1; most present stage 3-4 with terrible prognosis).↗
▶Ep 3 · 57:12
quoteRhabdoid tumors which we really have failed to treat very well. The only outcomes that we have reasonable outcomes are stage 1, but beyond that, and most of them present at stage 3 or stage 4, the outcomes are terrible.↗
▶Ep 3 · 57:46
clinicalBilateral Wilms tumors occur in 8-10% of cases. The strategy is preoperative chemotherapy to shrink tumors enough to allow at least one partial nephrectomy, avoiding dialysis.↗
▶Ep 3 · 58:40
epidemiologicalHistorical outcomes for bilateral Wilms were significantly worse than unilateral: 61% event-free survival and 80% overall survival for bilateral vs 88% event-free and 95% overall for unilateral.↗
▶Ep 3 · 59:13
clinicalThe recent COG bilateral Wilms study used VAD induction chemotherapy with imaging at 6 and 12 weeks. Maximum tumor response typically occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 3 · 1:00:29
clinicalIn typical-age children (<36 months) with bilateral renal tumors, biopsy is not mandatory as it is almost universally Wilms tumor. Of 250 patients enrolled in the recent study, only one had rhabdoid tumor (and the smaller lesion responded to therapy).↗
▶Ep 3 · 1:00:29
quoteWhen you look at children who present in the typical age under 36 months with bilateral renal tumors, it is almost universally Wilm's tumor.↗
▶Ep 3 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older children (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely. If biopsy is performed, both kidneys should be biopsied as there is discordant pathology in up to 20% of cases.↗
Peter's statements about Neuroblastoma110 statements
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 8 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 8 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 8 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 8 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 8 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 8 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 8 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 8 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 8 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 8 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 8 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 8 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 8 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 8 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 8 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 8 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 8 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 8 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 8 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 8 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 8 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 8 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 8 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 8 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 8 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 8 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 8 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 8 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 8 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 8 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 8 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 8 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 8 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 8 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 8 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 8 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 8 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 8 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 8 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 8 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 8 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 8 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 8 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 8 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 8 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 8 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 8 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 8 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 8 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 8 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 8 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 8 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 8 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 8 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 8 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 8 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 8 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 8 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 8 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 8 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 8 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 8 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 8 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 8 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 8 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 8 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 8 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 8 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 8 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 8 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 8 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 8 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 8 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 8 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 8 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 8 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 8 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 8 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 8 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 8 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 8 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 8 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 8 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 8 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 8 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 8 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 8 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 8 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 8 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 8 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 8 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 8 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 8 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 8 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 8 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 8 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 8 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 8 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 8 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 8 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 8 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 8 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 8 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 8 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 8 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 8 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 8 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 8 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
▶Ep 8 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Peter's statements about Pediatric Oncology103 statements
clinicalWilms tumors characteristically show a 'claw sign' on imaging where normal kidney is displaced into a horseshoe pattern, appearing to grab the mass coming out of it.↗
▶Ep 65 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing around them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 65 · 6:23
guidelineIn North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of children with renal tumors.↗
▶Ep 65 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 65 · 7:01
guidelinePreoperative chemotherapy is recommended if the tumor compromises the child's respiratory status, making them a poor operative candidate.↗
▶Ep 65 · 7:33
guidelineIf tumor extends into the inferior vena cava beyond the intrahepatic level (behind the liver or up to the atrium), preoperative chemotherapy is recommended.↗
▶Ep 65 · 7:57
guidelineMassive tumors that would require resection of large parts of liver or bowel should receive preoperative chemotherapy because the majority will respond.↗
▶Ep 65 · 8:21
guidelineIf the child has only one functioning kidney, preoperative chemotherapy is recommended to avoid nephrectomy.↗
▶Ep 65 · 8:33
guidelineChildren with bilateral renal tumors, Wilms tumor predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann), or multicentric tumors should not undergo primary nephrectomy.↗
▶Ep 65 · 10:38
clinicalComplication rates are higher if liver or bowel must be resected at the same time as the kidney.↗
▶Ep 65 · 11:18
guidelineIf gross tumor is left behind or the tumor is only biopsied, it is treated as a stage 3 abdominal tumor requiring 3-drug chemotherapy plus flank radiation.↗
▶Ep 65 · 14:19
quoteIf you feel that you would have to do a major liver or bowel resection at the same time, that is a situation in which we would not recommend doing a primary nephrectomy.↗
▶Ep 65 · 19:44
guidelineIn the COG staging system, treatment is determined by both local abdominal stage and disease stage.↗
▶Ep 65 · 20:01
guidelineStage 1 tumor is limited to the kidney, completely resected, with no capsular invasion, no rupture or biopsy prior to removal, no renal sinus vessel involvement, negative margins, and negative regional lymph nodes.↗
▶Ep 65 · 20:28
guidelineStage 2 tumor is completely resected with negative margins but extends beyond the kidney through capsular penetration, renal sinus soft tissue invasion, or blood vessel involvement outside the primary kidney.↗
▶Ep 65 · 21:00
guidelineStage 3 includes tumors that are biopsied with gross residual, have positive lymph nodes, penetrate the peritoneal surface with implants, have positive margins, have microscopic residual from intraoperative spill, cannot be completely resected, or must be removed in pieces.↗
▶Ep 65 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain.↗
▶Ep 65 · 22:06
guidelineStage 5 is bilateral renal tumor involvement.↗
▶Ep 65 · 23:08
guidelinePatients with stage 1 or 2 abdominal disease without lung metastases receive only 2-drug chemotherapy (vincristine and dactinomycin) for shorter duration with lower toxicity and significantly lower risk of late effects.↗
▶Ep 65 · 23:37
quoteThe two main factors that contribute to late effects are radiation and doxorubicin.↗
▶Ep 65 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy problems in females, hypertension, and cardiovascular disease, primarily caused by radiation and doxorubicin.↗
▶Ep 65 · 24:06
guidelineIf a child has stage 1 or 2 abdominal disease, they do not require abdominal radiation regardless of lung metastases status.↗
▶Ep 65 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 65 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer.↗
▶Ep 65 · 26:20
clinicalPulmonary radiation causes pneumonitis and long-term restrictive lung disease.↗
▶Ep 65 · 26:24
clinicalA recent COG study showed that approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks of chemotherapy do not need pulmonary radiation, with 80-85% remaining relapse-free.↗
▶Ep 65 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 65 · 30:31
guidelineIn the SIOP protocols, all patients start with chemotherapy without biopsy in most cases, using higher doses of two drugs, with evaluation at 4 and 8 weeks before proceeding to resection.↗
▶Ep 65 · 31:06
guidelineSIOP uses post-chemotherapy, post-nephrectomy classification into low risk, intermediate risk, and high risk based on percentage of blastemal components and presence of anaplasia.↗
▶Ep 65 · 35:11
clinicalSome children with Wilms tumor develop acquired von Willebrand disease; in the majority of cases it is meaningless, but a few case series report significant bleeding during surgery until the tumor is removed.↗
▶Ep 65 · 41:24
guidelinePositive surgical margins or tumor rupture (intraoperative or microscopic) automatically makes the patient stage 3.↗
▶Ep 65 · 42:31
guidelineTaking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 65 · 43:32
clinicalVery low-risk patients (stage I, <550g, <2 years, favorable histology) can be treated with surgery alone; 90-95% are cured without chemotherapy, and those who relapse have 100% survival with delayed chemotherapy.↗
▶Ep 65 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 65 · 46:38
guidelineIf IVC tumor thrombus extends into the renal vein but is not adherent and comes out in one piece, it is considered stage 2.↗
▶Ep 65 · 47:43
clinicalMajor complication rates (including mortality, blood transfusions, ICU stay) increase significantly when tumor extends beyond the infrahepatic IVC to the hepatic veins or atrium, with 26-30% major morbidity in primary resection cases.↗
▶Ep 65 · 51:28
quoteI've read 6000 operative notes. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 65 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and is associated with significantly worse outcomes regardless of stage.↗
▶Ep 65 · 55:23
epidemiologicalStage 1-2 patients with loss of heterozygosity at 1p and 16q have approximately 10% lower overall survival than those without these genetic changes.↗
▶Ep 65 · 55:45
clinicalStage 3-4 patients with loss of heterozygosity at 1p and 16q have approximately 18% lower overall survival and receive 5-drug regimen M chemotherapy.↗
▶Ep 65 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 65 · 58:51
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% with overall survival of only 80%.↗
▶Ep 65 · 58:51
epidemiologicalEvent-free survival for unilateral Wilms tumor is approximately 88% with overall survival of 95%.↗
▶Ep 65 · 59:37
clinicalThe maximum response of most children with Wilms tumor to chemotherapy occurs by 12 weeks.↗
▶Ep 65 · 1:00:31
clinicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor.↗
▶Ep 65 · 1:01:02
clinicalOpen biopsy to determine favorable vs. unfavorable histology in Wilms tumor is not very accurate initially.↗
▶Ep 65 · 1:01:12
epidemiologicalIn a recent COG bilateral Wilms study of 250 patients, only one patient who met enrollment criteria turned out to have rhabdoid tumor instead of Wilms tumor.↗
▶Ep 65 · 1:02:14
epidemiologicalDiscordant pathology occurs in up to 20% of bilateral Wilms tumor patients when both kidneys are biopsied.↗
Wilms Tumor: Audio Chapter
▶Ep 258 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 258 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 258 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 258 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 258 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 258 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 258 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 258 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 258 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 258 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 258 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 258 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 258 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 258 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 258 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 258 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 258 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 258 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 258 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 258 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 258 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 258 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 258 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 258 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 258 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 258 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 258 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 258 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 258 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 258 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 258 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 258 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 258 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 258 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 258 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 258 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 258 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 258 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 258 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 258 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 258 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 258 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 258 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 258 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 258 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 258 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 258 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 258 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 258 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 258 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 258 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 258 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 258 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 258 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 258 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Peter's statements about Sarcoma (Ewing/Rhabdo)55 statements
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 12 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 12 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 12 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 12 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 12 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 12 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 12 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 12 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 12 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 12 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 12 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 12 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 12 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 12 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 12 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 12 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 12 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 12 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 12 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 12 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 12 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 12 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 12 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 12 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 12 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 12 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 12 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 12 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 12 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 12 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 12 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 12 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 12 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 12 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 12 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 12 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 12 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 12 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 12 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 12 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 12 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 12 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 12 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 12 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 12 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 12 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 12 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 12 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 12 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 12 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 12 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 12 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 12 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 12 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Peter's statements about Soft Tissue Sarcoma (lymph nodes)55 statements
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 9 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 9 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 9 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 9 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 9 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 9 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 9 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 9 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 9 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 9 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 9 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 9 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 9 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 9 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 9 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 9 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 9 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 9 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 9 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 9 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 9 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 9 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 9 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 9 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 9 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 9 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 9 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 9 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 9 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 9 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 9 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 9 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 9 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 9 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 9 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 9 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 9 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 9 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 9 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 9 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 9 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 9 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 9 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 9 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 9 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 9 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 9 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 9 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 9 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 9 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 9 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 9 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 9 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 9 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Peter's statements about Wilms Tumor207 statements
clinicalWilms tumors characteristically show a 'claw sign' on imaging where normal kidney is displaced into a horseshoe pattern, appearing to grab the mass coming out of it.↗
▶Ep 2 · 3:49
clinicalWilms tumors characteristically show a 'claw sign' on imaging where normal kidney is displaced into a horseshoe pattern, appearing to grab the mass coming out of it.↗
▶Ep 2 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing around them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 2 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing around them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 2 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 2 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 2 · 6:23
guidelineIn North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of children with renal tumors.↗
▶Ep 2 · 6:23
guidelineIn North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of children with renal tumors.↗
▶Ep 2 · 7:01
guidelinePreoperative chemotherapy is recommended if the tumor compromises the child's respiratory status, making them a poor operative candidate.↗
▶Ep 2 · 7:01
guidelinePreoperative chemotherapy is recommended if the tumor compromises the child's respiratory status, making them a poor operative candidate.↗
▶Ep 2 · 7:33
guidelineIf tumor extends into the inferior vena cava beyond the intrahepatic level (behind the liver or up to the atrium), preoperative chemotherapy is recommended.↗
▶Ep 2 · 7:33
guidelineIf tumor extends into the inferior vena cava beyond the intrahepatic level (behind the liver or up to the atrium), preoperative chemotherapy is recommended.↗
▶Ep 2 · 7:57
guidelineMassive tumors that would require resection of large parts of liver or bowel should receive preoperative chemotherapy because the majority will respond.↗
▶Ep 2 · 7:57
guidelineMassive tumors that would require resection of large parts of liver or bowel should receive preoperative chemotherapy because the majority will respond.↗
▶Ep 2 · 8:21
guidelineIf the child has only one functioning kidney, preoperative chemotherapy is recommended to avoid nephrectomy.↗
▶Ep 2 · 8:21
guidelineIf the child has only one functioning kidney, preoperative chemotherapy is recommended to avoid nephrectomy.↗
▶Ep 2 · 8:33
guidelineChildren with bilateral renal tumors, Wilms tumor predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann), or multicentric tumors should not undergo primary nephrectomy.↗
▶Ep 2 · 8:33
guidelineChildren with bilateral renal tumors, Wilms tumor predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann), or multicentric tumors should not undergo primary nephrectomy.↗
▶Ep 2 · 10:38
clinicalComplication rates are higher if liver or bowel must be resected at the same time as the kidney.↗
▶Ep 2 · 10:38
clinicalComplication rates are higher if liver or bowel must be resected at the same time as the kidney.↗
▶Ep 2 · 11:18
guidelineIf gross tumor is left behind or the tumor is only biopsied, it is treated as a stage 3 abdominal tumor requiring 3-drug chemotherapy plus flank radiation.↗
▶Ep 2 · 11:18
guidelineIf gross tumor is left behind or the tumor is only biopsied, it is treated as a stage 3 abdominal tumor requiring 3-drug chemotherapy plus flank radiation.↗
▶Ep 2 · 14:19
quoteIf you feel that you would have to do a major liver or bowel resection at the same time, that is a situation in which we would not recommend doing a primary nephrectomy.↗
▶Ep 2 · 14:19
quoteIf you feel that you would have to do a major liver or bowel resection at the same time, that is a situation in which we would not recommend doing a primary nephrectomy.↗
▶Ep 2 · 19:44
guidelineIn the COG staging system, treatment is determined by both local abdominal stage and disease stage.↗
▶Ep 2 · 19:44
guidelineIn the COG staging system, treatment is determined by both local abdominal stage and disease stage.↗
▶Ep 2 · 20:01
guidelineStage 1 tumor is limited to the kidney, completely resected, with no capsular invasion, no rupture or biopsy prior to removal, no renal sinus vessel involvement, negative margins, and negative regional lymph nodes.↗
▶Ep 2 · 20:01
guidelineStage 1 tumor is limited to the kidney, completely resected, with no capsular invasion, no rupture or biopsy prior to removal, no renal sinus vessel involvement, negative margins, and negative regional lymph nodes.↗
▶Ep 2 · 20:28
guidelineStage 2 tumor is completely resected with negative margins but extends beyond the kidney through capsular penetration, renal sinus soft tissue invasion, or blood vessel involvement outside the primary kidney.↗
▶Ep 2 · 20:28
guidelineStage 2 tumor is completely resected with negative margins but extends beyond the kidney through capsular penetration, renal sinus soft tissue invasion, or blood vessel involvement outside the primary kidney.↗
▶Ep 2 · 21:00
guidelineStage 3 includes tumors that are biopsied with gross residual, have positive lymph nodes, penetrate the peritoneal surface with implants, have positive margins, have microscopic residual from intraoperative spill, cannot be completely resected, or must be removed in pieces.↗
▶Ep 2 · 21:00
guidelineStage 3 includes tumors that are biopsied with gross residual, have positive lymph nodes, penetrate the peritoneal surface with implants, have positive margins, have microscopic residual from intraoperative spill, cannot be completely resected, or must be removed in pieces.↗
▶Ep 2 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain.↗
▶Ep 2 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain.↗
▶Ep 2 · 22:06
guidelineStage 5 is bilateral renal tumor involvement.↗
▶Ep 2 · 22:06
guidelineStage 5 is bilateral renal tumor involvement.↗
▶Ep 2 · 23:08
guidelinePatients with stage 1 or 2 abdominal disease without lung metastases receive only 2-drug chemotherapy (vincristine and dactinomycin) for shorter duration with lower toxicity and significantly lower risk of late effects.↗
▶Ep 2 · 23:08
guidelinePatients with stage 1 or 2 abdominal disease without lung metastases receive only 2-drug chemotherapy (vincristine and dactinomycin) for shorter duration with lower toxicity and significantly lower risk of late effects.↗
▶Ep 2 · 23:37
quoteThe two main factors that contribute to late effects are radiation and doxorubicin.↗
▶Ep 2 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy problems in females, hypertension, and cardiovascular disease, primarily caused by radiation and doxorubicin.↗
▶Ep 2 · 23:37
quoteThe two main factors that contribute to late effects are radiation and doxorubicin.↗
▶Ep 2 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy problems in females, hypertension, and cardiovascular disease, primarily caused by radiation and doxorubicin.↗
▶Ep 2 · 24:06
guidelineIf a child has stage 1 or 2 abdominal disease, they do not require abdominal radiation regardless of lung metastases status.↗
▶Ep 2 · 24:06
guidelineIf a child has stage 1 or 2 abdominal disease, they do not require abdominal radiation regardless of lung metastases status.↗
▶Ep 2 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer.↗
▶Ep 2 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 2 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 2 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer.↗
▶Ep 2 · 26:20
clinicalPulmonary radiation causes pneumonitis and long-term restrictive lung disease.↗
▶Ep 2 · 26:20
clinicalPulmonary radiation causes pneumonitis and long-term restrictive lung disease.↗
▶Ep 2 · 26:24
clinicalA recent COG study showed that approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks of chemotherapy do not need pulmonary radiation, with 80-85% remaining relapse-free.↗
▶Ep 2 · 26:24
clinicalA recent COG study showed that approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks of chemotherapy do not need pulmonary radiation, with 80-85% remaining relapse-free.↗
▶Ep 2 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 2 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 2 · 30:31
guidelineIn the SIOP protocols, all patients start with chemotherapy without biopsy in most cases, using higher doses of two drugs, with evaluation at 4 and 8 weeks before proceeding to resection.↗
▶Ep 2 · 30:31
guidelineIn the SIOP protocols, all patients start with chemotherapy without biopsy in most cases, using higher doses of two drugs, with evaluation at 4 and 8 weeks before proceeding to resection.↗
▶Ep 2 · 31:06
guidelineSIOP uses post-chemotherapy, post-nephrectomy classification into low risk, intermediate risk, and high risk based on percentage of blastemal components and presence of anaplasia.↗
▶Ep 2 · 31:06
guidelineSIOP uses post-chemotherapy, post-nephrectomy classification into low risk, intermediate risk, and high risk based on percentage of blastemal components and presence of anaplasia.↗
▶Ep 2 · 35:11
clinicalSome children with Wilms tumor develop acquired von Willebrand disease; in the majority of cases it is meaningless, but a few case series report significant bleeding during surgery until the tumor is removed.↗
▶Ep 2 · 35:11
clinicalSome children with Wilms tumor develop acquired von Willebrand disease; in the majority of cases it is meaningless, but a few case series report significant bleeding during surgery until the tumor is removed.↗
▶Ep 2 · 41:24
guidelinePositive surgical margins or tumor rupture (intraoperative or microscopic) automatically makes the patient stage 3.↗
▶Ep 2 · 41:24
guidelinePositive surgical margins or tumor rupture (intraoperative or microscopic) automatically makes the patient stage 3.↗
▶Ep 2 · 42:31
guidelineTaking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 2 · 42:31
guidelineTaking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 2 · 43:32
clinicalVery low-risk patients (stage I, <550g, <2 years, favorable histology) can be treated with surgery alone; 90-95% are cured without chemotherapy, and those who relapse have 100% survival with delayed chemotherapy.↗
▶Ep 2 · 43:32
clinicalVery low-risk patients (stage I, <550g, <2 years, favorable histology) can be treated with surgery alone; 90-95% are cured without chemotherapy, and those who relapse have 100% survival with delayed chemotherapy.↗
▶Ep 2 · 43:48
quoteThese patients that no matter what you did, whether you treated them with surgery, whether you added 1 drug of chemotherapy, 2 drugs of chemotherapy, 3 drugs, radiation, that they had excellent overall survival.↗
▶Ep 2 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 2 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 2 · 46:38
guidelineIf IVC tumor thrombus extends into the renal vein but is not adherent and comes out in one piece, it is considered stage 2.↗
▶Ep 2 · 46:38
guidelineIf IVC tumor thrombus extends into the renal vein but is not adherent and comes out in one piece, it is considered stage 2.↗
▶Ep 2 · 47:43
clinicalMajor complication rates (including mortality, blood transfusions, ICU stay) increase significantly when tumor extends beyond the infrahepatic IVC to the hepatic veins or atrium, with 26-30% major morbidity in primary resection cases.↗
▶Ep 2 · 47:43
clinicalMajor complication rates (including mortality, blood transfusions, ICU stay) increase significantly when tumor extends beyond the infrahepatic IVC to the hepatic veins or atrium, with 26-30% major morbidity in primary resection cases.↗
▶Ep 2 · 51:28
quoteI've read 6000 operative notes. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 2 · 51:28
quoteI've read 6000 operative notes. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 2 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and is associated with significantly worse outcomes regardless of stage.↗
▶Ep 2 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and is associated with significantly worse outcomes regardless of stage.↗
▶Ep 2 · 55:23
epidemiologicalStage 1-2 patients with loss of heterozygosity at 1p and 16q have approximately 10% lower overall survival than those without these genetic changes.↗
▶Ep 2 · 55:23
epidemiologicalStage 1-2 patients with loss of heterozygosity at 1p and 16q have approximately 10% lower overall survival than those without these genetic changes.↗
▶Ep 2 · 55:45
clinicalStage 3-4 patients with loss of heterozygosity at 1p and 16q have approximately 18% lower overall survival and receive 5-drug regimen M chemotherapy.↗
▶Ep 2 · 55:45
clinicalStage 3-4 patients with loss of heterozygosity at 1p and 16q have approximately 18% lower overall survival and receive 5-drug regimen M chemotherapy.↗
▶Ep 2 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 2 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 2 · 58:51
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% with overall survival of only 80%.↗
▶Ep 2 · 58:51
epidemiologicalEvent-free survival for unilateral Wilms tumor is approximately 88% with overall survival of 95%.↗
▶Ep 2 · 58:51
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% with overall survival of only 80%.↗
▶Ep 2 · 58:51
epidemiologicalEvent-free survival for unilateral Wilms tumor is approximately 88% with overall survival of 95%.↗
▶Ep 2 · 59:37
clinicalThe maximum response of most children with Wilms tumor to chemotherapy occurs by 12 weeks.↗
▶Ep 2 · 59:37
clinicalThe maximum response of most children with Wilms tumor to chemotherapy occurs by 12 weeks.↗
▶Ep 2 · 1:00:31
clinicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor.↗
▶Ep 2 · 1:00:31
clinicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor.↗
▶Ep 2 · 1:01:02
clinicalOpen biopsy to determine favorable vs. unfavorable histology in Wilms tumor is not very accurate initially.↗
▶Ep 2 · 1:01:02
clinicalOpen biopsy to determine favorable vs. unfavorable histology in Wilms tumor is not very accurate initially.↗
▶Ep 2 · 1:01:12
epidemiologicalIn a recent COG bilateral Wilms study of 250 patients, only one patient who met enrollment criteria turned out to have rhabdoid tumor instead of Wilms tumor.↗
▶Ep 2 · 1:01:12
epidemiologicalIn a recent COG bilateral Wilms study of 250 patients, only one patient who met enrollment criteria turned out to have rhabdoid tumor instead of Wilms tumor.↗
▶Ep 2 · 1:02:14
epidemiologicalDiscordant pathology occurs in up to 20% of bilateral Wilms tumor patients when both kidneys are biopsied.↗
▶Ep 2 · 1:02:14
epidemiologicalDiscordant pathology occurs in up to 20% of bilateral Wilms tumor patients when both kidneys are biopsied.↗
Wilms Tumor: Audio Chapter
▶Ep 10 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 10 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 10 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 10 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 10 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 10 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 10 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 10 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 10 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 10 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 10 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 10 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 10 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 10 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 10 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 10 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 10 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 10 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 10 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 10 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 10 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 10 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 10 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 10 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 10 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 10 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 10 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 10 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 10 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 10 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 10 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 10 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 10 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 10 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 10 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 10 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 10 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 10 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 10 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 10 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 10 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 10 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 10 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 10 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 10 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 10 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 10 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 10 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 10 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 10 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 10 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 10 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 10 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 10 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 10 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 10 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 10 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 10 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 10 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 10 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 10 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 10 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 10 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 10 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 10 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 10 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 10 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 10 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 10 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 10 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 10 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 10 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 10 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 10 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 10 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 10 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 10 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 10 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 10 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 10 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 10 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 10 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 10 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 10 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 10 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 10 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 10 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 10 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 10 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 10 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 10 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 10 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 10 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 10 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 10 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 10 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 10 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 10 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 10 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 10 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 10 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 10 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 10 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 10 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 10 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 10 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 10 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 10 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 10 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
▶Ep 10 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
Summaries Peter gave as host
· 3 summaries
Recaps of other experts' statements, not Peter's own clinical position.
Summaries Peter gave as host · Nephroblastoma1 summary
host summaryPeter Ehrlich summarizing a resource: These patients that no matter what you did, whether you treated them with surgery, whether you added 1 drug of chemotherapy, 2 drugs of chemotherapy, 3 drugs, radiation, that they had excellent overall survival.↗
Summaries Peter gave as host · Pediatric Oncology1 summary
host summaryPeter Ehrlich summarizing a resource: These patients that no matter what you did, whether you treated them with surgery, whether you added 1 drug of chemotherapy, 2 drugs of chemotherapy, 3 drugs, radiation, that they had excellent overall survival.↗
Summaries Peter gave as host · Wilms Tumor1 summary
host summaryPeter Ehrlich summarizing a resource: These patients that no matter what you did, whether you treated them with surgery, whether you added 1 drug of chemotherapy, 2 drugs of chemotherapy, 3 drugs, radiation, that they had excellent overall survival.↗
