# Nephroblastoma — GCMD Library living collection Everything in the library about nephroblastoma — built automatically from dossiers that name it. Updated: n/a · 3 episodes · 129 cited statements ## Episodes ### In-Depth Reviews - [Wilms Tumor: Audio Chapter](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926) — podcast · 1:04:03 · [machine version](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926.md) - [Wilms Tumor: Audio Chapter](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487) — podcast · 1:04:03 · [machine version](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487.md) - [Wilms Tumor](https://library.globalcastmd.com/watch/wilms-tumor-13443) — podcast · 1:04:03 · [machine version](https://library.globalcastmd.com/watch/wilms-tumor-13443.md) ## Chapters - [0:00](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=0) Introduction and Case Presentation (Ep 1) - [1:54](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=114) Differential Diagnosis and Imaging Features (Ep 1) - [4:50](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=290) Workup and Indications for Primary Surgery vs. Preoperative Chemotherapy (Ep 1) - [8:53](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=533) Staging Systems and Treatment Implications (Ep 1) - [14:56](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=896) Biopsy Technique and Central Line Placement (Ep 1) - [19:34](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1174) COG Staging System Details and Treatment Stratification (Ep 1) - [24:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1456) Management of Pulmonary Metastases (Ep 1) - [27:24](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1644) COG vs. SIOP Treatment Philosophies (Ep 1) - [30:31](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1831) Surgical Technique: Preoperative Preparation and Approach (Ep 1) - [37:55](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2275) Operative Technique: Nephroureterectomy and Lymph Node Sampling (Ep 1) - [41:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2476) Surgical Margins and Tumor Rupture (Ep 1) - [43:29](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2609) Very Low-Risk Patients: Surgery-Only Cohort (Ep 1) - [45:27](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2727) Management of IVC Tumor Thrombus (Ep 1) - [51:56](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3116) Pathology and Risk Stratification (Ep 1) - [56:41](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3401) Bilateral Wilms Tumor Management (Ep 1) - [62:34](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3754) Closing Remarks (Ep 1) - [0:00](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=0) Introduction and Differential Diagnosis of Pediatric Renal Masses (Ep 2) - [4:50](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=290) Initial Workup and Indications for Primary Surgery vs. Biopsy (Ep 2) - [8:53](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=533) Preoperative Chemotherapy Indications and Biopsy Technique (Ep 2) - [15:25](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=925) COG Staging System: Abdominal and Disease Stage (Ep 2) - [23:02](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1382) Treatment Based on Stage and Management of Pulmonary Metastases (Ep 2) - [28:51](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1731) COG vs. SIOP Treatment Philosophies (Ep 2) - [36:03](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2163) Surgical Technique: Positioning, Incision, and Resection Principles (Ep 2) - [41:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2476) Surgical Margins, Spillage, and Adherent Structures (Ep 2) - [45:56](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2756) Management of IVC Tumor Thrombus (Ep 2) - [52:29](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3149) Pathologic Variants and Risk Stratification (Ep 2) - [57:36](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3456) Bilateral Wilms Tumor Management (Ep 2) - [63:44](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3824) Closing Remarks (Ep 2) - [0:00](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=0) Introduction and Expert Background (Ep 3) - [1:31](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=91) Differential Diagnosis and Imaging Features (Ep 3) - [4:50](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=290) Initial Workup and Indications for Primary Surgery vs Chemotherapy (Ep 3) - [8:53](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=533) Biopsy Indications and Staging Systems (Ep 3) - [15:26](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=926) Management of Pulmonary Metastases (Ep 3) - [19:34](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1174) COG Staging System Details (Ep 3) - [24:16](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1456) Chemotherapy Regimens and Late Effects (Ep 3) - [28:51](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1731) Surgical Technique and Preoperative Preparation (Ep 3) - [34:35](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2075) Surgical Margins, Staging Implications, and Adherent Structures (Ep 3) - [43:29](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2609) Surgery-Only Treatment for Very Low Risk Patients (Ep 3) - [45:27](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2727) Management of IVC Tumor Thrombus (Ep 3) - [52:29](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3149) Pathology Results and Treatment Implications (Ep 3) ## Statements Every statement is attributed, typed, and timestamped; the link is the citation. - In North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling). — Peter Ehrlich (guideline) [Ep 2 · 6:23](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=383) - Preoperative 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. — Peter Ehrlich (guideline) [Ep 2 · 7:01](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=421) - The 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor. — Peter Ehrlich (clinical) [Ep 2 · 3:49](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=229) - Wilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels. — Peter Ehrlich (clinical) [Ep 2 · 4:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=256) - If 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. — Peter Ehrlich (clinical) [Ep 2 · 10:24](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=624) - Biopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation. — Peter Ehrlich (guideline) [Ep 2 · 11:18](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=678) - Evidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy. — Peter Ehrlich (clinical) [Ep 2 · 14:33](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=873) - For 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. — Peter Ehrlich (guideline) [Ep 2 · 17:19](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1039) - COG 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. — Peter Ehrlich (guideline) [Ep 2 · 20:00](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1200) - COG 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. — Peter Ehrlich (guideline) [Ep 2 · 20:28](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1228) - COG 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. — Peter Ehrlich (guideline) [Ep 2 · 21:00](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1260) - COG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement. — Peter Ehrlich (guideline) [Ep 2 · 21:58](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1318) - Stage 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. — Peter Ehrlich (guideline) [Ep 2 · 23:08](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1388) - If 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). — Peter Ehrlich (clinical) [Ep 2 · 24:15](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1455) - Recent 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. — Peter Ehrlich (clinical) [Ep 2 · 26:24](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1584) - Fifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect. — Peter Ehrlich (epidemiological) [Ep 2 · 26:08](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1568) - For 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. — Peter Ehrlich (clinical) [Ep 2 · 28:09](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1689) - SIOP 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). — Peter Ehrlich (guideline) [Ep 2 · 30:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1816) - In 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. — Peter Ehrlich (clinical) [Ep 2 · 31:19](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1879) - Outcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches. — Peter Ehrlich (clinical) [Ep 2 · 33:58](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2038) - Some Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds. — Peter Ehrlich (clinical) [Ep 2 · 34:46](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2086) - Acquired 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. — Peter Ehrlich (clinical) [Ep 2 · 35:11](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2111) - Right-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. — Peter Ehrlich (clinical) [Ep 2 · 36:25](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2185) - Wilms 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. — Peter Ehrlich (clinical) [Ep 2 · 42:31](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2551) - The 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. — Peter Ehrlich (clinical) [Ep 2 · 32:50](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=1970) - Patients 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. — Peter Ehrlich (clinical) [Ep 2 · 54:41](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3281) - Patients 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. — Peter Ehrlich (guideline) [Ep 2 · 55:07](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3307) - IVC tumor extension is not a negative prognostic factor if the tumor is completely resected. — Peter Ehrlich (clinical) [Ep 2 · 46:27](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2787) - Major 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. — Peter Ehrlich (clinical) [Ep 2 · 48:49](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2929) - Favorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology. — Peter Ehrlich (clinical) [Ep 2 · 52:39](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3159) - Unfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each. — Peter Ehrlich (clinical) [Ep 2 · 55:58](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3358) - Clear 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. — Peter Ehrlich (clinical) [Ep 2 · 57:01](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3421) - Renal cell carcinoma in children has no good therapy, particularly for metastatic disease. — Peter Ehrlich (clinical) [Ep 2 · 56:51](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3411) - Bilateral Wilms tumors occur in 8-10% of all children with Wilms tumor. — Peter Ehrlich (epidemiological) [Ep 2 · 57:46](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3466) - The 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. — Peter Ehrlich (guideline) [Ep 2 · 58:07](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3487) - Event-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%. — Peter Ehrlich (epidemiological) [Ep 2 · 58:40](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3520) - Maximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response. — Peter Ehrlich (clinical) [Ep 2 · 59:37](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3577) - In 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. — Peter Ehrlich (epidemiological) [Ep 2 · 60:31](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3631) - Biopsy 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. — Peter Ehrlich (guideline) [Ep 2 · 61:56](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3716) - If biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients. — Peter Ehrlich (clinical) [Ep 2 · 62:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=3736) - A 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. — Peter Ehrlich (clinical) [Ep 2 · 43:32](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-3487?t=2612) - In North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of children with renal tumors. — Peter Ehrlich (guideline) [Ep 1 · 6:23](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=383) - Preoperative chemotherapy is recommended if the tumor compromises the child's respiratory status, making them a poor operative candidate. — Peter Ehrlich (guideline) [Ep 1 · 7:01](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=421) - If tumor extends into the inferior vena cava beyond the intrahepatic level (behind the liver or up to the atrium), preoperative chemotherapy is recommended. — Peter Ehrlich (guideline) [Ep 1 · 7:33](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=453) - Massive tumors that would require resection of large parts of liver or bowel should receive preoperative chemotherapy because the majority will respond. — Peter Ehrlich (guideline) [Ep 1 · 7:57](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=477) - If the child has only one functioning kidney, preoperative chemotherapy is recommended to avoid nephrectomy. — Peter Ehrlich (guideline) [Ep 1 · 8:21](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=501) - Children with bilateral renal tumors, Wilms tumor predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann), or multicentric tumors should not undergo primary nephrectomy. — Peter Ehrlich (guideline) [Ep 1 · 8:33](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=513) - Complication rates are higher if liver or bowel must be resected at the same time as the kidney. — Peter Ehrlich (clinical) [Ep 1 · 10:38](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=638) - If 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. — Peter Ehrlich (guideline) [Ep 1 · 11:18](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=678) - The maximum response of most children with Wilms tumor to chemotherapy occurs by 12 weeks. — Peter Ehrlich (clinical) [Ep 1 · 59:37](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3577) - In the COG staging system, treatment is determined by both local abdominal stage and disease stage. — Peter Ehrlich (guideline) [Ep 1 · 19:44](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1184) - Stage 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. — Peter Ehrlich (guideline) [Ep 1 · 20:01](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1201) - Stage 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. — Peter Ehrlich (guideline) [Ep 1 · 20:28](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1228) - Stage 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. — Peter Ehrlich (guideline) [Ep 1 · 21:00](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1260) - Stage 4 is hematogenous metastasis to lung, liver, bone, or brain. — Peter Ehrlich (guideline) [Ep 1 · 21:58](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1318) - Stage 5 is bilateral renal tumor involvement. — Peter Ehrlich (guideline) [Ep 1 · 22:06](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1326) - Patients 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. — Peter Ehrlich (guideline) [Ep 1 · 23:08](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1388) - The 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. — Peter Ehrlich (clinical) [Ep 1 · 23:37](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1417) - If a child has stage 1 or 2 abdominal disease, they do not require abdominal radiation regardless of lung metastases status. — Peter Ehrlich (guideline) [Ep 1 · 24:06](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1446) - A 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. — Peter Ehrlich (clinical) [Ep 1 · 26:24](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1584) - Fifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer. — Peter Ehrlich (epidemiological) [Ep 1 · 26:08](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1568) - Pulmonary radiation causes pneumonitis and long-term restrictive lung disease. — Peter Ehrlich (clinical) [Ep 1 · 26:20](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1580) - In 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. — Peter Ehrlich (guideline) [Ep 1 · 30:31](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1831) - SIOP uses post-chemotherapy, post-nephrectomy classification into low risk, intermediate risk, and high risk based on percentage of blastemal components and presence of anaplasia. — Peter Ehrlich (guideline) [Ep 1 · 31:06](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=1866) - Some 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. — Peter Ehrlich (clinical) [Ep 1 · 35:11](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2111) - Wilms 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. — Peter Ehrlich (clinical) [Ep 1 · 3:49](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=229) - Wilms tumor tends to push structures out of the way rather than growing around them, whereas neuroblastoma grows around structures like blood vessels. — Peter Ehrlich (clinical) [Ep 1 · 4:16](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=256) - Positive surgical margins or tumor rupture (intraoperative or microscopic) automatically makes the patient stage 3. — Peter Ehrlich (guideline) [Ep 1 · 41:24](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2484) - Taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided. — Peter Ehrlich (guideline) [Ep 1 · 42:31](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2551) - Very 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. — Peter Ehrlich (clinical) [Ep 1 · 43:32](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2612) - IVC tumor extension is not a negative prognostic factor if the tumor is completely resected. — Peter Ehrlich (clinical) [Ep 1 · 46:14](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2774) - If IVC tumor thrombus extends into the renal vein but is not adherent and comes out in one piece, it is considered stage 2. — Peter Ehrlich (guideline) [Ep 1 · 46:38](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2798) - Major 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. — Peter Ehrlich (clinical) [Ep 1 · 47:43](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=2863) - Loss of heterozygosity at both 1p and 16q occurs in 5-7% of patients and is associated with significantly worse outcomes regardless of stage. — Peter Ehrlich (clinical) [Ep 1 · 54:41](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3281) - Stage 1-2 patients with loss of heterozygosity at 1p and 16q have approximately 10% lower overall survival than those without these genetic changes. — Peter Ehrlich (epidemiological) [Ep 1 · 55:23](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3323) - Stage 3-4 patients with loss of heterozygosity at 1p and 16q have approximately 18% lower overall survival and receive 5-drug regimen M chemotherapy. — Peter Ehrlich (clinical) [Ep 1 · 55:45](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3345) - Bilateral Wilms tumors occur in 8-10% of all children with Wilms tumor. — Peter Ehrlich (epidemiological) [Ep 1 · 57:46](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3466) - Event-free survival for unilateral Wilms tumor is approximately 88% with overall survival of 95%. — Peter Ehrlich (epidemiological) [Ep 1 · 58:51](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3531) - Event-free survival for bilateral Wilms tumor on NWTS-5 was 61% with overall survival of only 80%. — Peter Ehrlich (epidemiological) [Ep 1 · 58:51](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3531) - In children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. — Peter Ehrlich (clinical) [Ep 1 · 60:31](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3631) - Open biopsy to determine favorable vs. unfavorable histology in Wilms tumor is not very accurate initially. — Peter Ehrlich (clinical) [Ep 1 · 61:02](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3662) - In 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. — Peter Ehrlich (epidemiological) [Ep 1 · 61:12](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3672) - Discordant pathology occurs in up to 20% of bilateral Wilms tumor patients when both kidneys are biopsied. — Peter Ehrlich (epidemiological) [Ep 1 · 62:14](https://library.globalcastmd.com/watch/wilms-tumor-audio-chapter-926?t=3734) - In North America, the Children's Oncology Group recommends primary nephrectomy and ureterectomy with lymph node sampling for the majority of renal tumors in children. — Peter Ehrlich (guideline) [Ep 3 · 6:23](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=383) - Preoperative chemotherapy is recommended when tumor extends into the IVC beyond the infrahepatic level, as complication rates are higher with primary surgery in these cases. — Peter Ehrlich (guideline) [Ep 3 · 7:33](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=453) - Preoperative 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. — Peter Ehrlich (guideline) [Ep 3 · 7:57](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=477) - Children with bilateral Wilms tumors, solitary functioning kidney, or genetic predisposition syndromes (Wagr, Denys-Drash, Beckwith-Wiedemann) should receive preoperative chemotherapy rather than primary nephrectomy. — Peter Ehrlich (guideline) [Ep 3 · 8:33](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=513) - In 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). — Peter Ehrlich (guideline) [Ep 3 · 15:55](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=955) - For 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. — Peter Ehrlich (guideline) [Ep 3 · 17:19](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1039) - A 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. — Peter Ehrlich (guideline) [Ep 3 · 17:46](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1066) - COG 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. — Peter Ehrlich (guideline) [Ep 3 · 19:44](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1184) - Stage 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. — Peter Ehrlich (guideline) [Ep 3 · 20:28](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1228) - Stage 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. — Peter Ehrlich (guideline) [Ep 3 · 21:00](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1260) - Stage 4 is hematogenous metastasis to lung, liver, bone, or brain. Stage 5 is bilateral renal involvement. — Peter Ehrlich (guideline) [Ep 3 · 21:58](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1318) - A 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. — Peter Ehrlich (guideline) [Ep 3 · 22:20](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1340) - Stage 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. — Peter Ehrlich (guideline) [Ep 3 · 23:08](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1388) - The 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. — Peter Ehrlich (clinical) [Ep 3 · 23:37](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1417) - If 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. — Peter Ehrlich (guideline) [Ep 3 · 24:15](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1455) - A 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. — Peter Ehrlich (clinical) [Ep 3 · 26:02](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1562) - Fifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, making radiation avoidance in responders a significant benefit. — Peter Ehrlich (epidemiological) [Ep 3 · 26:08](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1568) - For 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. — Peter Ehrlich (guideline) [Ep 3 · 27:54](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1674) - All patients with lung lesions presumed to be metastatic disease start with three-drug chemotherapy. — Peter Ehrlich (guideline) [Ep 3 · 29:50](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1790) - The 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. — Peter Ehrlich (guideline) [Ep 3 · 30:15](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1815) - SIOP 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. — Peter Ehrlich (guideline) [Ep 3 · 31:06](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1866) - In 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. — Peter Ehrlich (clinical) [Ep 3 · 31:42](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1902) - Outcomes for stage 1 and 2 patients are essentially identical between COG and SIOP approaches. — Peter Ehrlich (clinical) [Ep 3 · 33:54](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2034) - There 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. — Peter Ehrlich (clinical) [Ep 3 · 32:45](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=1965) - Preoperative assessment should include hemoglobin (some tumors rupture internally causing anemia), PT/PTT (acquired von Willebrand disease can occur, rarely causing significant bleeding), and electrolytes. — Peter Ehrlich (guideline) [Ep 3 · 34:46](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2086) - Surgical 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. — Peter Ehrlich (guideline) [Ep 3 · 37:24](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2244) - Lymph 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. — Peter Ehrlich (guideline) [Ep 3 · 39:49](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2389) - Taking 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. — Peter Ehrlich (opinion) [Ep 3 · 40:19](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2419) - Positive surgical margins automatically make the tumor stage 3. Microscopic rupture through the capsule that is only visible on pathology also creates stage 3 disease. — Peter Ehrlich (guideline) [Ep 3 · 41:24](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2484) - Taking 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. — Peter Ehrlich (clinical) [Ep 3 · 42:31](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2551) - Very 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. — Peter Ehrlich (clinical) [Ep 3 · 43:32](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2612) - IVC 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. — Peter Ehrlich (clinical) [Ep 3 · 46:14](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2774) - For 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. — Peter Ehrlich (guideline) [Ep 3 · 46:49](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2809) - Tumors extending above the infrahepatic IVC have significantly higher complication rates (26-30% major morbidity) with primary surgery and should receive preoperative chemotherapy. — Peter Ehrlich (clinical) [Ep 3 · 47:43](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=2863) - After 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. — Peter Ehrlich (clinical) [Ep 3 · 51:28](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3088) - Favorable 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. — Peter Ehrlich (clinical) [Ep 3 · 52:39](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3159) - Stage 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. — Peter Ehrlich (guideline) [Ep 3 · 53:45](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3225) - Loss 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. — Peter Ehrlich (clinical) [Ep 3 · 54:25](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3265) - Patients 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). — Peter Ehrlich (guideline) [Ep 3 · 55:23](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3323) - Unfavorable 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. — Peter Ehrlich (guideline) [Ep 3 · 55:58](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3358) - Other 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). — Peter Ehrlich (clinical) [Ep 3 · 56:51](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3411) - Bilateral 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. — Peter Ehrlich (clinical) [Ep 3 · 57:46](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3466) - Historical 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. — Peter Ehrlich (epidemiological) [Ep 3 · 58:40](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3520) - The 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. — Peter Ehrlich (clinical) [Ep 3 · 59:13](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3553) - In 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). — Peter Ehrlich (clinical) [Ep 3 · 60:29](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3629) - Biopsy 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 Ehrlich (guideline) [Ep 3 · 61:56](https://library.globalcastmd.com/watch/wilms-tumor-13443?t=3716) ## Changelog - Oct 15: 3 items added automatically --- Educational content from recorded physician discussions — not medical advice. Cite the canonical URL or the ?t= deep link. Policy: https://library.globalcastmd.com/ai