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Nephroblastoma
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Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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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, Wilms Tumor: Audio Chapter · 31:06
Wilms Tumor: Audio Chapter
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An interactive discussion about Wilms tumor between Todd Ponsky, MD, Avraham Schlager, and Peter Ehrlich, MD.Dr. Peter Ehrlich is a professor of pediatrics surgery at the University of Michigan C.S. Mott Children’s Hospital who is also a vi
podcast1:04:03 · Jan 2019
Wilms Tumor: Audio Chapter
Listen →
An interactive discussion about Wilms tumor between Todd Ponsky, MD, Avraham Schlager, and Peter Ehrlich, MD.Dr. Peter Ehrlich is a professor of pediatrics surgery at the University of Michigan C.S. Mott Children’s Hospital who is also a vi
podcast1:04:03 · Jan 2021
Wilms Tumor
Listen →
An interactive discussion about Wilms tumor between Todd Ponsky, MD, Avraham Schlager, and Peter Ehrlich, MD. Dr. Peter Ehrlich is a professor of pediatrics su
podcast1:04:03 · Jun 2026
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Wilms Tumor: Audio Chapter
In North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).
guidelinePeter Ehrlich6:23 ↗
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.
guidelinePeter Ehrlich7:01 ↗
The 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.
clinicalPeter Ehrlich3:49 ↗
Wilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.
clinicalPeter Ehrlich4:16 ↗
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.
clinicalPeter Ehrlich10:24 ↗
Biopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.
guidelinePeter Ehrlich11:18 ↗
Evidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.
clinicalPeter Ehrlich14:33 ↗
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.
guidelinePeter Ehrlich17:19 ↗
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.
guidelinePeter Ehrlich20:00 ↗
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.
guidelinePeter Ehrlich20:28 ↗
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.
guidelinePeter Ehrlich21:00 ↗
COG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.
guidelinePeter Ehrlich21:58 ↗
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.
guidelinePeter Ehrlich23:08 ↗
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).
clinicalPeter Ehrlich24:15 ↗
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.
clinicalPeter Ehrlich26:24 ↗
Fifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.
epidemiologicalPeter Ehrlich26:08 ↗
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.
clinicalPeter Ehrlich28:09 ↗
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).
guidelinePeter Ehrlich30:16 ↗
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.
clinicalPeter Ehrlich31:19 ↗
Outcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.
clinicalPeter Ehrlich33:58 ↗
Some Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.
clinicalPeter Ehrlich34:46 ↗
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.
clinicalPeter Ehrlich35:11 ↗
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.
clinicalPeter Ehrlich36:25 ↗
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.
clinicalPeter Ehrlich42:31 ↗
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.
clinicalPeter Ehrlich32:50 ↗
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.
clinicalPeter Ehrlich54:41 ↗
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.
guidelinePeter Ehrlich55:07 ↗
IVC tumor extension is not a negative prognostic factor if the tumor is completely resected.
clinicalPeter Ehrlich46:27 ↗
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.
clinicalPeter Ehrlich48:49 ↗
Favorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.
clinicalPeter Ehrlich52:39 ↗
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