Wilms Tumor: Audio Chapter
With Dr. Peter Ehrlich · hosted by Dr. Todd Ponsky · StayCurrentMD
Part of
Wilms Tumor 20 items
Cued at 51:28 · stops at 52:13 · press play
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
Podcast
Wilms Tumor: Audio Chapter
64 min · Published Jun 2017
Video
Wilms Tumor
Published May 2020
Podcast
Topics in 10: Wilms Tumor
12 min · Published Aug 2019
Video
Update Course Rewind: Updates in Wilms Management 2024
4 min · Published Sep 2025
Video
Wilms Tumor Protocol Violations: Practice Gap discussion at Update Course 2018
9 min · Published Aug 2018
Podcast
Neuroblastoma
56 min · Published Aug 2019
Only a few other public items share this expert — go deeper there →
Video
Pediatric Surgical Oncology Research Collaborative (PSORC): Studying Rare Pediatric Tumors
56 s · Published May 2026
Video
Update Course Rewind 2025: Hirschsprung + ARM: Rare but Real
1 min · Published May 2026
Video
Update Course Rewind 2025: Hirschsprung + ARM: Rare but Real
1 min · Published May 2026
Video
Pooling Patients to Study Rare Pediatric Tumors: An Introduction to PSORC
56 s · Published May 2026
Video
The fetal frontier: A review of current and emerging fetal therapies for genetic diseases
44 s · Published May 2026
Video
Indocyanine green assists with sentinel lymph node mapping in pediatric and adolescent patients
1 min · Published May 2026
What the experts said
In North America, the preferred approach for most renal tumors is primary total nephrectomy with ureterectomy and lymph node sampling
Preoperative 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
Tumors between 13–15 centimeters or larger have higher risk of rupture and may warrant consideration of preoperative chemotherapy
Biopsy is recommended when giving preoperative chemotherapy because imaging cannot distinguish between Wilms tumor, rhabdoid tumor, clear cell sarcoma, or determine favorable vs unfavorable histology
Core needle biopsy requires at least 10 cores, with accuracy increasing between 10 and 20 cores; fine needle aspiration cannot diagnose anaplasia
Stage 1 is tumor limited to kidney, completely resected, no capsular invasion, vessels not involved, negative margins and lymph nodes
Stage 2 is completely resected tumor with regional extension such as capsular penetration or renal sinus invasion, but negative lymph nodes and margins
Stage 3 includes biopsied tumors with gross residual, positive lymph nodes, peritoneal penetration, positive margins, microscopic residual from spillage, or piecemeal removal
Stage 4 is hematogenous metastasis to lung, liver, bone, or brain; stage 5 is bilateral renal involvement
Stage 1 or 2 abdominal disease without lung metastases receives only two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation
The main late effects of concern are renal failure, second malignancies, pregnancy problems, hypertension, and cardiovascular disease, primarily driven by radiation and doxorubicin exposure
If 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
Fifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer
In COG protocols, approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks can avoid pulmonary radiation without compromising survival
SIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with response assessment at 4 and 8 weeks before surgery
SIOP classifies patients post-treatment as low risk (complete necrosis), intermediate risk (based on blastema percentage), or high risk (predominantly blastema or anaplasia)
In stage 3 disease, the main prognostic factor is lymph node status, followed by loss of heterozygosity at 1p and 16q, and 1q gain
Attempting resection and having to bail out with biopsy does not worsen prognosis compared to empiric biopsy for stage 3 disease
Some Wilms tumor patients develop acquired von Willebrand disease; while usually clinically insignificant, rare cases have had significant intraoperative bleeding
Right-sided tumors can distort anatomy leading to potential duodenal injury, superior mesenteric artery injury, or IVC injury
The 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
Adrenalectomy is not necessary; there are no reports of adrenal insufficiency and adrenal vein tumor presence does not correlate with outcomes
Lymph node sampling should aim for at least 5–6 nodes from the renal hilum and para-aortic or paracaval regions
Taking 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
Very 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
The 10% of very low-risk patients who relapse after surgery alone have 100% salvage survival with chemotherapy
IVC tumor extension is not a negative prognostic factor if the tumor can be completely resected
For 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
Major complication rates including mortality, transfusions, and ICU stay increase significantly when IVC thrombus extends above the infrahepatic level
In the largest series of IVC thrombus extending beyond infrahepatic cava treated with primary surgery, there was 26–30% major morbidity and some mortality
Intraoperative ultrasound is used to assess the superior extent of IVC thrombus and determine if partial or complete caval occlusion is needed for resection
Loss 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
Patients 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
Unfavorable histology is classified as focal or diffuse anaplasia based on the number of high-power fields showing anaplastic features
Clear cell sarcoma of the kidney has reasonable treatment outcomes particularly for low stages, while rhabdoid tumors have terrible outcomes except for stage 1
Bilateral Wilms tumors occur in 8–10% of all Wilms tumor cases
The strategy for bilateral Wilms is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy on at least one kidney, avoiding dialysis
Historical outcomes for bilateral Wilms tumor were significantly worse than unilateral: 61% event-free survival and 80% overall survival compared to 88% and 95% respectively
The COG bilateral Wilms study used VAD induction chemotherapy with response assessment at 6 and 12 weeks, as maximum response typically occurs by 12 weeks
In 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
Biopsy 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
When biopsying bilateral disease, both kidneys should be biopsied as there is discordant pathology in up to 20% of cases