LONG TERM OUTCOME OF PATIENTS WITH BILATERAL WILMS TREATED WITH RENAL SALVAGE SURGERY

Background: To cure children with bilateral wilms disease and maintain adequate renal function is a surgical challenge, as successful renal salvage is technically demanding. The aim of this study was to review all patients with bilateral wilms treated with partial nephrectomy from one institution. M...

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Published in:Pediatrics (Evanston) 1999-09, Vol.104 (3), p.817-817
Main Authors: Kubiak, Rainer, Wilcox, Duncan T, Duffy, Patrick G, Ransley, Philip G
Format: Article
Language:English
Subjects:
Pediatrics
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Summary:Background: To cure children with bilateral wilms disease and maintain adequate renal function is a surgical challenge, as successful renal salvage is technically demanding. The aim of this study was to review all patients with bilateral wilms treated with partial nephrectomy from one institution. Methods: This study is a retrospective review of all patients with histologically proven bilateral wilms disease treated with a partial nephrectomy, in one institution between April 1972 and October 1997. Results: Nineteen patients were identified, adequate follow up data was available in 18. The median age at diagnosis was 20.9 months (range 7.9-65), the median follow up was 57 months (range 12.5-297). All patients were treated with a combination of surgery and chemotherapy. Of the 36 kidneys, 8 had a nephrectomy and 22 a renal salvage procedure. Twenty patients had an in situ partial nephrectomy and 2 bench surgery with subsequent autotransplantation. The histological margins, following surgery, were clear in 19, unknown in 2 and not clear in 1 ... The renal function in these 18 patients was normal in 14, abnormal but not requiring dialysis in 2 and 2 developed renal failure. The causes of renal failure were pre-existing glomerulonephritis and a chemotherapy induced tubularopathy. Twelve of the 18 children are alive and tumour free at follow up (66%). Four children died from distant metastases and 2 from renal failure. None of the salvaged kidneys had detected local recurrence. Conclusions: These results confirm that renal salvage procedures, in combination with chemotherapy, are a safe and effective way of treating children with bilateral wilms disease. In addition, renal salvage surgery can maintain satisfactory renal function in the vast majority of these patients, without increasing local recurrence.
ISSN:0031-4005