There is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred
There is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred
There is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred
There is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred
There is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred
Todd, 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.
clinicalThe claw sign on CT scan—normal kidney displaced into horseshoe pattern with tumor in the center—is classic for Wilms tumor↗
▶Ep 24 · 4:16
clinicalWilms tumors tend to push structures out of the way rather than growing around them, unlike neuroblastoma↗
▶Ep 24 · 4:16
quoteWilm's tumor also tends 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 24 · 6:23
guidelineIn North America, the preferred approach for resectable renal tumors is primary nephrectomy and ureterectomy with lymph node sampling↗
▶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 · 7:33
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic vena cava↗
▶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 · 14:33
clinicalTumors between 13-15 centimeters or larger have higher risk of rupture and may warrant preoperative chemotherapy↗
▶Ep 24 · 18:03
guidelineCore needle biopsy should include 10-20 cores to increase diagnostic accuracy; fine needle aspiration cannot diagnose anaplasia↗
guidelineStage 1 and 2 Wilms tumor patients receive only two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks without radiation↗
▶Ep 24 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy complications, hypertension, and cardiovascular disease, primarily from radiation and doxorubicin↗
▶Ep 24 · 24:15
clinicalA patient with lung metastases but stage 1-2 abdominal disease does not require abdominal radiation if primary tumor is completely resected with negative lymph nodes↗
▶Ep 24 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer as a late effect↗
▶Ep 24 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 24 · 26:44
clinicalApproximately 40% of patients with pulmonary metastases achieve complete response by 6 weeks of chemotherapy and can avoid pulmonary radiation↗
▶Ep 24 · 27:00
quoteSo those kids completely avoided pulmonary radiation and the Toxicity without any effect on either their event free or overall survival.↗
▶Ep 24 · 30:31
guidelineSIOP protocols use preoperative chemotherapy for all patients with post-treatment staging based on blastemal predominance↗
▶Ep 24 · 32:45
clinicalLymph node positivity is the main prognostic factor in stage 3 disease↗
▶Ep 24 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients and may rarely cause significant intraoperative bleeding↗
▶Ep 24 · 39:49
guidelineMinimum lymph node sampling should be 5-6 nodes, though more than one may be better based on secondary evidence↗
▶Ep 24 · 41:24
guidelinePositive surgical margins automatically make a tumor stage 3↗
▶Ep 24 · 42:31
clinicalTaking a rim of adherent diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided↗
▶Ep 24 · 43:41
clinicalPatients with tumors less than 550g, stage 1, favorable histology, and age under 2 years can be treated with surgery alone with greater than 95% survival↗
▶Ep 24 · 46:14
clinicalIVC tumor thrombus extension is not a negative prognostic factor if completely resected↗
▶Ep 24 · 48:49
clinicalMajor complication rate for tumors extending beyond infrahepatic IVC operated upfront is 26-30% with documented mortality↗
▶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 · 51:43
quoteThere is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred↗
▶Ep 24 · 52:52
clinicalFavorable histology Wilms tumor has three components: blastemal, stromal, and epithelial; tumors with only two components are still considered favorable↗
▶Ep 24 · 53:13
clinicalIn COG protocols, the amount of blastema in favorable histology tumors has never correlated with outcome↗
▶Ep 24 · 53:56
guidelineStage 2 patients receive vincristine and dactinomycin for 19 weeks; stage 3 patients receive DD4A regimen (adding doxorubicin) for 25 weeks↗
▶Ep 24 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and predicts 10% lower survival in stage 1-2 and 18% lower survival in stage 3-4↗
▶Ep 24 · 56:51
clinicalRenal cell carcinoma is the second most common renal tumor in children, with no effective therapy for metastatic disease↗
▶Ep 24 · 57:27
clinicalRhabdoid tumors have terrible outcomes except for stage 1; most present at stage 3 or 4↗
▶Ep 24 · 58:51
epidemiologicalBilateral Wilms tumor occurs in 8-10% of cases with historically lower survival: 61% event-free and 80% overall compared to 88% and 95% for unilateral disease↗
▶Ep 24 · 59:37
clinicalMaximum tumor response to chemotherapy typically occurs by 12 weeks, with early response at 6 weeks predicting late response↗
▶Ep 24 · 1:02:14
clinicalIn bilateral Wilms tumor, there is discordant pathology between kidneys in up to 20% of cases↗
clinicalThe claw sign on CT scan—normal kidney displaced into horseshoe pattern with tumor in the center—is classic for Wilms tumor↗
▶Ep 8 · 4:16
clinicalWilms tumors tend to push structures out of the way rather than growing around them, unlike neuroblastoma↗
▶Ep 8 · 4:16
quoteWilm's tumor also tends 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 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 renal tumors is primary nephrectomy and ureterectomy with lymph node sampling↗
▶Ep 8 · 7:33
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic vena cava↗
▶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 · 14:33
clinicalTumors between 13-15 centimeters or larger have higher risk of rupture and may warrant preoperative chemotherapy↗
▶Ep 8 · 18:03
guidelineCore needle biopsy should include 10-20 cores to increase diagnostic accuracy; fine needle aspiration cannot diagnose anaplasia↗
guidelineStage 1 and 2 Wilms tumor patients receive only two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks without radiation↗
▶Ep 8 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy complications, hypertension, and cardiovascular disease, primarily from radiation and doxorubicin↗
▶Ep 8 · 24:15
clinicalA patient with lung metastases but stage 1-2 abdominal disease does not require abdominal radiation if primary tumor is completely resected with negative lymph nodes↗
▶Ep 8 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer as a late effect↗
▶Ep 8 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 8 · 26:44
clinicalApproximately 40% of patients with pulmonary metastases achieve complete response by 6 weeks of chemotherapy and can avoid pulmonary radiation↗
▶Ep 8 · 27:00
quoteSo those kids completely avoided pulmonary radiation and the Toxicity without any effect on either their event free or overall survival.↗
▶Ep 8 · 30:31
guidelineSIOP protocols use preoperative chemotherapy for all patients with post-treatment staging based on blastemal predominance↗
▶Ep 8 · 32:45
clinicalLymph node positivity is the main prognostic factor in stage 3 disease↗
▶Ep 8 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients and may rarely cause significant intraoperative bleeding↗
▶Ep 8 · 39:49
guidelineMinimum lymph node sampling should be 5-6 nodes, though more than one may be better based on secondary evidence↗
▶Ep 8 · 41:24
guidelinePositive surgical margins automatically make a tumor stage 3↗
▶Ep 8 · 42:31
clinicalTaking a rim of adherent diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided↗
▶Ep 8 · 43:41
clinicalPatients with tumors less than 550g, stage 1, favorable histology, and age under 2 years can be treated with surgery alone with greater than 95% survival↗
▶Ep 8 · 46:14
clinicalIVC tumor thrombus extension is not a negative prognostic factor if completely resected↗
▶Ep 8 · 48:49
clinicalMajor complication rate for tumors extending beyond infrahepatic IVC operated upfront is 26-30% with documented mortality↗
▶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 · 51:43
quoteThere is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred↗
▶Ep 8 · 52:52
clinicalFavorable histology Wilms tumor has three components: blastemal, stromal, and epithelial; tumors with only two components are still considered favorable↗
▶Ep 8 · 53:13
clinicalIn COG protocols, the amount of blastema in favorable histology tumors has never correlated with outcome↗
▶Ep 8 · 53:56
guidelineStage 2 patients receive vincristine and dactinomycin for 19 weeks; stage 3 patients receive DD4A regimen (adding doxorubicin) for 25 weeks↗
▶Ep 8 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and predicts 10% lower survival in stage 1-2 and 18% lower survival in stage 3-4↗
▶Ep 8 · 56:51
clinicalRenal cell carcinoma is the second most common renal tumor in children, with no effective therapy for metastatic disease↗
▶Ep 8 · 57:27
clinicalRhabdoid tumors have terrible outcomes except for stage 1; most present at stage 3 or 4↗
▶Ep 8 · 58:51
epidemiologicalBilateral Wilms tumor occurs in 8-10% of cases with historically lower survival: 61% event-free and 80% overall compared to 88% and 95% for unilateral disease↗
▶Ep 8 · 59:37
clinicalMaximum tumor response to chemotherapy typically occurs by 12 weeks, with early response at 6 weeks predicting late response↗
▶Ep 8 · 1:02:14
clinicalIn bilateral Wilms tumor, there is discordant pathology between kidneys in up to 20% of cases↗
clinicalThe claw sign on CT scan—normal kidney displaced into horseshoe pattern with tumor in the center—is classic for Wilms tumor↗
▶Ep 12 · 4:16
quoteWilm's tumor also tends 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 12 · 4:16
clinicalWilms tumors tend to push structures out of the way rather than growing around them, unlike neuroblastoma↗
▶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 renal tumors is primary nephrectomy and ureterectomy with lymph node sampling↗
▶Ep 12 · 7:33
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic vena cava↗
▶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 · 14:33
clinicalTumors between 13-15 centimeters or larger have higher risk of rupture and may warrant preoperative chemotherapy↗
▶Ep 12 · 18:03
guidelineCore needle biopsy should include 10-20 cores to increase diagnostic accuracy; fine needle aspiration cannot diagnose anaplasia↗
guidelineStage 1 and 2 Wilms tumor patients receive only two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks without radiation↗
▶Ep 12 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy complications, hypertension, and cardiovascular disease, primarily from radiation and doxorubicin↗
▶Ep 12 · 24:15
clinicalA patient with lung metastases but stage 1-2 abdominal disease does not require abdominal radiation if primary tumor is completely resected with negative lymph nodes↗
▶Ep 12 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer as a late effect↗
▶Ep 12 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 12 · 26:44
clinicalApproximately 40% of patients with pulmonary metastases achieve complete response by 6 weeks of chemotherapy and can avoid pulmonary radiation↗
▶Ep 12 · 27:00
quoteSo those kids completely avoided pulmonary radiation and the Toxicity without any effect on either their event free or overall survival.↗
▶Ep 12 · 30:31
guidelineSIOP protocols use preoperative chemotherapy for all patients with post-treatment staging based on blastemal predominance↗
▶Ep 12 · 32:45
clinicalLymph node positivity is the main prognostic factor in stage 3 disease↗
▶Ep 12 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients and may rarely cause significant intraoperative bleeding↗
▶Ep 12 · 39:49
guidelineMinimum lymph node sampling should be 5-6 nodes, though more than one may be better based on secondary evidence↗
▶Ep 12 · 41:24
guidelinePositive surgical margins automatically make a tumor stage 3↗
▶Ep 12 · 42:31
clinicalTaking a rim of adherent diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided↗
▶Ep 12 · 43:41
clinicalPatients with tumors less than 550g, stage 1, favorable histology, and age under 2 years can be treated with surgery alone with greater than 95% survival↗
▶Ep 12 · 46:14
clinicalIVC tumor thrombus extension is not a negative prognostic factor if completely resected↗
▶Ep 12 · 48:49
clinicalMajor complication rate for tumors extending beyond infrahepatic IVC operated upfront is 26-30% with documented mortality↗
▶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 · 51:43
quoteThere is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred↗
▶Ep 12 · 52:52
clinicalFavorable histology Wilms tumor has three components: blastemal, stromal, and epithelial; tumors with only two components are still considered favorable↗
▶Ep 12 · 53:13
clinicalIn COG protocols, the amount of blastema in favorable histology tumors has never correlated with outcome↗
▶Ep 12 · 53:56
guidelineStage 2 patients receive vincristine and dactinomycin for 19 weeks; stage 3 patients receive DD4A regimen (adding doxorubicin) for 25 weeks↗
▶Ep 12 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and predicts 10% lower survival in stage 1-2 and 18% lower survival in stage 3-4↗
▶Ep 12 · 56:51
clinicalRenal cell carcinoma is the second most common renal tumor in children, with no effective therapy for metastatic disease↗
▶Ep 12 · 57:27
clinicalRhabdoid tumors have terrible outcomes except for stage 1; most present at stage 3 or 4↗
▶Ep 12 · 58:51
epidemiologicalBilateral Wilms tumor occurs in 8-10% of cases with historically lower survival: 61% event-free and 80% overall compared to 88% and 95% for unilateral disease↗
▶Ep 12 · 59:37
clinicalMaximum tumor response to chemotherapy typically occurs by 12 weeks, with early response at 6 weeks predicting late response↗
▶Ep 12 · 1:02:14
clinicalIn bilateral Wilms tumor, there is discordant pathology between kidneys in up to 20% of cases↗
clinicalThe claw sign on CT scan—normal kidney displaced into horseshoe pattern with tumor in the center—is classic for Wilms tumor↗
▶Ep 9 · 4:16
clinicalWilms tumors tend to push structures out of the way rather than growing around them, unlike neuroblastoma↗
▶Ep 9 · 4:16
quoteWilm's tumor also tends 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 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 · 6:23
guidelineIn North America, the preferred approach for resectable renal tumors is primary nephrectomy and ureterectomy with lymph node sampling↗
▶Ep 9 · 7:33
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic vena cava↗
▶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 · 14:33
clinicalTumors between 13-15 centimeters or larger have higher risk of rupture and may warrant preoperative chemotherapy↗
▶Ep 9 · 18:03
guidelineCore needle biopsy should include 10-20 cores to increase diagnostic accuracy; fine needle aspiration cannot diagnose anaplasia↗
guidelineStage 1 and 2 Wilms tumor patients receive only two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks without radiation↗
▶Ep 9 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy complications, hypertension, and cardiovascular disease, primarily from radiation and doxorubicin↗
▶Ep 9 · 24:15
clinicalA patient with lung metastases but stage 1-2 abdominal disease does not require abdominal radiation if primary tumor is completely resected with negative lymph nodes↗
▶Ep 9 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer as a late effect↗
▶Ep 9 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 9 · 26:44
clinicalApproximately 40% of patients with pulmonary metastases achieve complete response by 6 weeks of chemotherapy and can avoid pulmonary radiation↗
▶Ep 9 · 27:00
quoteSo those kids completely avoided pulmonary radiation and the Toxicity without any effect on either their event free or overall survival.↗
▶Ep 9 · 30:31
guidelineSIOP protocols use preoperative chemotherapy for all patients with post-treatment staging based on blastemal predominance↗
▶Ep 9 · 32:45
clinicalLymph node positivity is the main prognostic factor in stage 3 disease↗
▶Ep 9 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients and may rarely cause significant intraoperative bleeding↗
▶Ep 9 · 39:49
guidelineMinimum lymph node sampling should be 5-6 nodes, though more than one may be better based on secondary evidence↗
▶Ep 9 · 41:24
guidelinePositive surgical margins automatically make a tumor stage 3↗
▶Ep 9 · 42:31
clinicalTaking a rim of adherent diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided↗
▶Ep 9 · 43:41
clinicalPatients with tumors less than 550g, stage 1, favorable histology, and age under 2 years can be treated with surgery alone with greater than 95% survival↗
▶Ep 9 · 46:14
clinicalIVC tumor thrombus extension is not a negative prognostic factor if completely resected↗
▶Ep 9 · 48:49
clinicalMajor complication rate for tumors extending beyond infrahepatic IVC operated upfront is 26-30% with documented mortality↗
▶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 · 51:43
quoteThere is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred↗
▶Ep 9 · 52:52
clinicalFavorable histology Wilms tumor has three components: blastemal, stromal, and epithelial; tumors with only two components are still considered favorable↗
▶Ep 9 · 53:13
clinicalIn COG protocols, the amount of blastema in favorable histology tumors has never correlated with outcome↗
▶Ep 9 · 53:56
guidelineStage 2 patients receive vincristine and dactinomycin for 19 weeks; stage 3 patients receive DD4A regimen (adding doxorubicin) for 25 weeks↗
▶Ep 9 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and predicts 10% lower survival in stage 1-2 and 18% lower survival in stage 3-4↗
▶Ep 9 · 56:51
clinicalRenal cell carcinoma is the second most common renal tumor in children, with no effective therapy for metastatic disease↗
▶Ep 9 · 57:27
clinicalRhabdoid tumors have terrible outcomes except for stage 1; most present at stage 3 or 4↗
▶Ep 9 · 58:51
epidemiologicalBilateral Wilms tumor occurs in 8-10% of cases with historically lower survival: 61% event-free and 80% overall compared to 88% and 95% for unilateral disease↗
▶Ep 9 · 59:37
clinicalMaximum tumor response to chemotherapy typically occurs by 12 weeks, with early response at 6 weeks predicting late response↗
▶Ep 9 · 1:02:14
clinicalIn bilateral Wilms tumor, there is discordant pathology between kidneys in up to 20% of cases↗
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 most renal tumors is primary total nephrectomy with ureterectomy and lymph node sampling↗
▶Ep 2 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic level, if tumor is so large it impairs respiratory status, if major liver or bowel resection would be required, or if only one functioning kidney exists↗
▶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 · 12:19
quoteIf you just often Wilmston which will push up to the liver and some people will take a little rim of that just to make sure they have a clear margin, that's completely different.↗
▶Ep 2 · 14:33
clinicalTumors between 13–15 centimeters or larger have higher risk of rupture and may warrant consideration of preoperative chemotherapy↗
▶Ep 2 · 15:55
guidelineBiopsy is recommended when giving preoperative chemotherapy because imaging cannot distinguish between Wilms tumor, rhabdoid tumor, clear cell sarcoma, or determine favorable vs unfavorable histology↗
▶Ep 2 · 17:33
clinicalCore needle biopsy requires at least 10 cores, with accuracy increasing between 10 and 20 cores; fine needle aspiration cannot diagnose anaplasia↗
▶Ep 2 · 20:00
guidelineStage 1 is tumor limited to kidney, completely resected, no capsular invasion, vessels not involved, negative margins and lymph nodes↗
▶Ep 2 · 20:28
guidelineStage 2 is completely resected tumor with regional extension such as capsular penetration or renal sinus invasion, but negative lymph nodes and margins↗
▶Ep 2 · 21:00
guidelineStage 3 includes biopsied tumors with gross residual, positive lymph nodes, peritoneal penetration, positive margins, microscopic residual from spillage, or piecemeal removal↗
▶Ep 2 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain; stage 5 is bilateral renal involvement↗
▶Ep 2 · 23:08
guidelineStage 1 or 2 abdominal disease without lung metastases receives only two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation↗
▶Ep 2 · 23:37
clinicalThe main late effects of concern are renal failure, second malignancies, pregnancy problems, hypertension, and cardiovascular disease, primarily driven by radiation and doxorubicin exposure↗
▶Ep 2 · 24:15
guidelineIf a patient has lung metastases but stage 1 or 2 abdominal disease after primary nephrectomy, they avoid abdominal radiation even though they receive three-drug chemotherapy for the lung disease↗
▶Ep 2 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer↗
▶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
clinicalIn COG protocols, approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks can avoid pulmonary radiation without compromising survival↗
▶Ep 2 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with response assessment at 4 and 8 weeks before surgery↗
▶Ep 2 · 31:19
guidelineSIOP classifies patients post-treatment as low risk (complete necrosis), intermediate risk (based on blastema percentage), or high risk (predominantly blastema or anaplasia)↗
▶Ep 2 · 32:45
clinicalIn stage 3 disease, the main prognostic factor is lymph node status, followed by loss of heterozygosity at 1p and 16q, and 1q gain↗
▶Ep 2 · 33:34
clinicalAttempting resection and having to bail out with biopsy does not worsen prognosis compared to empiric biopsy for stage 3 disease↗
▶Ep 2 · 35:11
clinicalSome Wilms tumor patients develop acquired von Willebrand disease; while usually clinically insignificant, rare cases have had significant intraoperative bleeding↗
▶Ep 2 · 36:25
clinicalRight-sided tumors can distort anatomy leading to potential duodenal injury, superior mesenteric artery injury, or IVC injury↗
▶Ep 2 · 37:19
clinicalThe classic Wilms tumor surgical approach involves transverse or subcostal incision, mobilizing the kidney onto its pedicle, then identifying and dividing the ureter distally, followed by hilar vessel control↗
▶Ep 2 · 39:23
clinicalAdrenalectomy is not necessary; there are no reports of adrenal insufficiency and adrenal vein tumor presence does not correlate with outcomes↗
▶Ep 2 · 39:49
guidelineLymph node sampling should aim for at least 5–6 nodes from the renal hilum and para-aortic or paracaval regions↗
▶Ep 2 · 42:31
clinicalTaking a rim of diaphragm or small piece of liver to avoid violating tumor capsule does not upstage the tumor if the tumor itself is not divided↗
▶Ep 2 · 43:32
clinicalVery low-risk patients (less than 2 years old, tumor less than 550 grams, stage 1, favorable histology) can be treated with surgery alone with greater than 95% survival↗
▶Ep 2 · 45:01
clinicalThe 10% of very low-risk patients who relapse after surgery alone have 100% salvage survival with chemotherapy↗
▶Ep 2 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if the tumor can be completely resected↗
▶Ep 2 · 47:07
clinicalFor infrahepatic IVC thrombus, the ideal technique is to mobilize the kidney, ligate the renal artery, make a small nick in the renal vein, and slide the thrombus out in one piece↗
▶Ep 2 · 47:43
clinicalMajor complication rates including mortality, transfusions, and ICU stay increase significantly when IVC thrombus extends above the infrahepatic level↗
▶Ep 2 · 48:49
epidemiologicalIn the largest series of IVC thrombus extending beyond infrahepatic cava treated with primary surgery, there was 26–30% major morbidity and some mortality↗
▶Ep 2 · 49:47
clinicalIntraoperative ultrasound is used to assess the superior extent of IVC thrombus and determine if partial or complete caval occlusion is needed for resection↗
▶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 · 52:07
quoteThree months ago I had to take out a kit, and they had all collaterals and the easiest thing I did, I Took out the whole cava because he wasn't using it↗
▶Ep 2 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5–7% of patients and is associated with 10% worse survival in stage 1–2 and 18% worse in stage 3–4↗
▶Ep 2 · 55:23
guidelinePatients with loss of heterozygosity at 1p and 16q receive intensified treatment: stage 1–2 get three drugs instead of two, stage 3–4 get five-drug regimen M↗
▶Ep 2 · 55:58
clinicalUnfavorable histology is classified as focal or diffuse anaplasia based on the number of high-power fields showing anaplastic features↗
▶Ep 2 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes particularly for low stages, while rhabdoid tumors have terrible outcomes except for stage 1↗
▶Ep 2 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8–10% of all Wilms tumor cases↗
▶Ep 2 · 58:07
guidelineThe strategy for bilateral Wilms is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy on at least one kidney, avoiding dialysis↗
▶Ep 2 · 58:51
epidemiologicalHistorical outcomes for bilateral Wilms tumor were significantly worse than unilateral: 61% event-free survival and 80% overall survival compared to 88% and 95% respectively↗
▶Ep 2 · 59:19
guidelineThe COG bilateral Wilms study used VAD induction chemotherapy with response assessment at 6 and 12 weeks, as maximum response typically occurs by 12 weeks↗
▶Ep 2 · 1:00:29
clinicalIn typical bilateral Wilms presentations (under 36 months, classic imaging), biopsy is not required as it is almost universally Wilms tumor; only 1 of 250 enrolled patients had rhabdoid tumor↗
▶Ep 2 · 1:01:56
guidelineBiopsy is recommended for atypical bilateral presentations: older children (8–10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely↗
▶Ep 2 · 1:02:14
clinicalWhen biopsying bilateral disease, both kidneys should be biopsied as there is discordant pathology in up to 20% of cases↗
Wilms Tumor: Audio Chapter
▶Ep 10 · 3:49
clinicalThe claw sign on CT scan—normal kidney displaced into horseshoe pattern with tumor in the center—is classic for Wilms tumor↗
▶Ep 10 · 4:16
quoteWilm's tumor also tends 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 10 · 4:16
clinicalWilms tumors tend to push structures out of the way rather than growing around them, unlike neuroblastoma↗
▶Ep 10 · 6:23
guidelineIn North America, the preferred approach for resectable renal tumors is primary 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 · 7:33
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic vena cava↗
▶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 · 14:33
clinicalTumors between 13-15 centimeters or larger have higher risk of rupture and may warrant preoperative chemotherapy↗
▶Ep 10 · 18:03
guidelineCore needle biopsy should include 10-20 cores to increase diagnostic accuracy; fine needle aspiration cannot diagnose anaplasia↗
guidelineStage 1 and 2 Wilms tumor patients receive only two-drug chemotherapy (vincristine and dactinomycin) for 19 weeks without radiation↗
▶Ep 10 · 23:37
clinicalThe main late effects of Wilms tumor treatment are renal failure, second malignancies, pregnancy complications, hypertension, and cardiovascular disease, primarily from radiation and doxorubicin↗
▶Ep 10 · 24:15
clinicalA patient with lung metastases but stage 1-2 abdominal disease does not require abdominal radiation if primary tumor is completely resected with negative lymph nodes↗
▶Ep 10 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer as a late effect↗
▶Ep 10 · 26:08
quote15% of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 10 · 26:44
clinicalApproximately 40% of patients with pulmonary metastases achieve complete response by 6 weeks of chemotherapy and can avoid pulmonary radiation↗
▶Ep 10 · 27:00
quoteSo those kids completely avoided pulmonary radiation and the Toxicity without any effect on either their event free or overall survival.↗
▶Ep 10 · 30:31
guidelineSIOP protocols use preoperative chemotherapy for all patients with post-treatment staging based on blastemal predominance↗
▶Ep 10 · 32:45
clinicalLymph node positivity is the main prognostic factor in stage 3 disease↗
▶Ep 10 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients and may rarely cause significant intraoperative bleeding↗
▶Ep 10 · 39:49
guidelineMinimum lymph node sampling should be 5-6 nodes, though more than one may be better based on secondary evidence↗
▶Ep 10 · 41:24
guidelinePositive surgical margins automatically make a tumor stage 3↗
▶Ep 10 · 42:31
clinicalTaking a rim of adherent diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided↗
▶Ep 10 · 43:41
clinicalPatients with tumors less than 550g, stage 1, favorable histology, and age under 2 years can be treated with surgery alone with greater than 95% survival↗
▶Ep 10 · 46:14
clinicalIVC tumor thrombus extension is not a negative prognostic factor if completely resected↗
▶Ep 10 · 48:49
clinicalMajor complication rate for tumors extending beyond infrahepatic IVC operated upfront is 26-30% with documented mortality↗
▶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 · 51:43
quoteThere is what people say you're supposed to do, and then there's The reality and you know, after reading 6000 and not seeing that, I'm not sure that some of these things that are described from historically actually occurred↗
▶Ep 10 · 52:52
clinicalFavorable histology Wilms tumor has three components: blastemal, stromal, and epithelial; tumors with only two components are still considered favorable↗
▶Ep 10 · 53:13
clinicalIn COG protocols, the amount of blastema in favorable histology tumors has never correlated with outcome↗
▶Ep 10 · 53:56
guidelineStage 2 patients receive vincristine and dactinomycin for 19 weeks; stage 3 patients receive DD4A regimen (adding doxorubicin) for 25 weeks↗
▶Ep 10 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and predicts 10% lower survival in stage 1-2 and 18% lower survival in stage 3-4↗
▶Ep 10 · 56:51
clinicalRenal cell carcinoma is the second most common renal tumor in children, with no effective therapy for metastatic disease↗
▶Ep 10 · 57:27
clinicalRhabdoid tumors have terrible outcomes except for stage 1; most present at stage 3 or 4↗
▶Ep 10 · 58:51
epidemiologicalBilateral Wilms tumor occurs in 8-10% of cases with historically lower survival: 61% event-free and 80% overall compared to 88% and 95% for unilateral disease↗
▶Ep 10 · 59:37
clinicalMaximum tumor response to chemotherapy typically occurs by 12 weeks, with early response at 6 weeks predicting late response↗
▶Ep 10 · 1:02:14
clinicalIn bilateral Wilms tumor, there is discordant pathology between kidneys in up to 20% of cases↗
