2024 Update Course - The New Approaches for Wilms Tumor - Michael Nightingale, Bhargava Mullapudi, & Justin Huntington
With Dr. Justin Huntington & Dr. Michael Nightingale
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What the experts said
Loss of heterozygosity at 1P and 16Q requires addition of doxorubicin to chemotherapy regimen
Loss of heterozygosity 1P/16Q shows higher recurrence rates in Wilms tumor
Adverse biologic factors show worse prognosis in stage 2 but not significantly in stage 1 favorable histology Wilms
Age and tumor nephrectomy weight are not associated with event-free survival in current COG enrollment data
Future protocols may allow older patients (>2 years) and higher tumor weights (>550g) with favorable biology to avoid chemotherapy
IVC thrombus below hepatic veins can be managed with upfront resection; above hepatic veins requires preoperative chemotherapy
COG approach avoids biopsy upfront for typical Wilms presentations
Biopsy indicated if chemotherapy response is inadequate, to rule out anaplasia which changes chemotherapy protocol
Vascular isolation for IVC thrombus may require sternotomy for supradiaphragmatic IVC control at right atrium level
Pringle maneuver and retrocaval IVC control below renals needed for safe IVC thrombus resection
All SIOP patients under 6 months of age are treated with surgical intent without preoperative chemotherapy
Nephron-sparing surgery in unilateral non-syndromic Wilms carries oncologic risk that must be weighed against renal preservation benefits
Only 1% of unilateral Wilms patients develop end-stage renal failure, making benefit of nephron-sparing surgery difficult to demonstrate
Hypertension and cardiovascular disease in Wilms survivors may be consequence of chemotherapy rather than nephron loss
Preoperative chemotherapy shrinks tumors and makes them more robust and rubbery, facilitating minimally invasive surgery
Two main risks of minimally invasive surgery for Wilms are tumor rupture and inadequate lymph node dissection
Aortic-caval lymph nodes are the most important region to sample in Wilms tumor
Minimum of 6 lymph nodes required, but aim for 7-10 nodes in SIOP protocols
Microscopic lymph node metastases can disappear with preoperative chemotherapy, necessitating higher node counts
ICG (indocyanine green) is emerging as adjunct to identify lymph node location during Wilms dissection
For atrial IVC thrombus, diaphragm can be mobilized from suprahepatic cava to create pericardial window for clamp placement without bypass
Bypass only needed for atrial thrombus if attached to cardiac valves