Nephron sparing surgery for synchronous bilateral nephroblastoma involving the renal hilus

Bilateral nephroblastoma involving the renal hilus represents a significant surgical challenge. Different operative strategies have been proposed for this condition. We analyzed the outcome of simultaneous bilateral partial nephrectomy for complex stage V nephroblastoma.

We retrospectively analyzed patients with bilateral nephroblastoma involving the renal hilus operated on at our institution between 2002 and 2008. We assessed patient data and surgical and oncologic outcomes.

We analyzed data from 5 patients with a median age of 27 months at surgery (range 13 to 58). Two children had additional pulmonary metastases. Patients were treated according to the International Society of Pediatric Oncology 2001/German Society of Pediatric Oncology and Hematology protocol. All children underwent synchronous bilateral nephron sparing surgery (longitudinal partial nephrectomy or enucleation) of the central tumors. Median operating time was 182 minutes (range 129 to 291), with vascular exclusion performed in 4 patients (7 to 25 minutes). Complications consisted of urinary leakage in 2 cases. Postoperative renal function was unimpaired in 4 patients and 1 patient had Wilms tumor/aniridia/genitourinary malformations/retardation syndrome with glomerulopathy. One patient with pulmonary metastases and blastemal histology had multiple pulmonary relapses and died due to pulmonary progressive disease. Four patients are alive without evidence of disease at a median followup of 45 months (range 44 to 73).

Surgery for central stage V nephroblastoma is possible with good functional and oncologic outcomes. The single stage approach is safe, provided that operating and treating physicians have sufficient expertise with such conditions. The ultimate goal is to avoid tumor nephrectomy.