A 5-year-old child is found on CT to have a large retroperitoneal mass with calcifications arising from the kidney. It shows areas of necrosis. The tumour shows loss of heterozygosity at chromosome 11p13 (WT1 locus). This diagnosis is best treated with which combination per current COG/SIOP protocols for stage III disease?
- A Primary surgery followed by flank radiotherapy and chemotherapy (actinomycin D, vincristine, doxorubicin) ✓
- B Surgery alone followed by surveillance
- C Neoadjuvant chemotherapy (SIOP: actinomycin-D + vincristine) then surgery then adjuvant chemoradiotherapy
- D Radical nephrectomy followed by ifosfamide/etoposide/carboplatin
Correct answer: A. Primary surgery followed by flank radiotherapy and chemotherapy (actinomycin D, vincristine, doxorubicin)
Explanation
This is Wilms' tumour (nephroblastoma). In the COG (North American) approach, upfront nephrectomy is performed for localised stages, followed by adjuvant chemotherapy and, for stage III, flank/abdominal radiotherapy. SIOP (European) uses neoadjuvant chemotherapy before surgery to reduce tumour bulk and spillage risk. Stage III includes positive lymph nodes, positive margins, or peritoneal seeding. COG regimen for stage III includes actinomycin D, vincristine, and doxorubicin plus abdominal radiation.
Reference: Bailey & Love's Short Practice of Surgery, 27th ed.
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Written and medically reviewed by the StethoPrep medical team.