Bilateral Wilms' tumor: a clinicopathologic review

Area of Science:

• Pediatric Oncology
• Nephrology
• Surgical Oncology

Background:

• Bilateral Wilms tumor presents a unique challenge in pediatric oncology, requiring a balance between complete tumor resection and preservation of vital renal function.
• This condition typically affects younger children compared to unilateral Wilms tumor, necessitating tailored treatment strategies.

Purpose of the Study:

• To review the surgical and oncological management of bilateral Wilms tumor over a 15-year period.
• To evaluate the outcomes, including survival and renal function, following conservative surgical approaches and chemotherapy.

Main Methods:

• Retrospective review of 46 Wilms tumor cases diagnosed at Princess Margaret Hospital.
• Analysis of diagnostic imaging, surgical procedures (nephrectomy, biopsy), pathological findings, and treatment protocols (chemotherapy, radiotherapy).

Main Results:

• Eight cases of bilateral Wilms tumor were identified, with a median age of 1.1 years.
• Conservative management involving bilateral biopsy and delayed resection, combined with vincristine and actinomycin chemotherapy, resulted in excellent survival and renal function in most patients.
• Pathological review confirmed true bilateral disease in four patients, while others had unilateral tumors with contralateral nephrogenic rests.

Conclusions:

• Conservative surgical management combined with low-toxicity chemotherapy is highly effective for bilateral Wilms tumor.
• This approach ensures excellent long-term renal function and survival, making it the preferred strategy for most pediatric patients.
• Accurate pathological assessment is crucial for differentiating true bilateral disease from unilateral tumors with nephrogenic rests.