Non-Wilms’ Renal Tumors In Childhood

Abstract

Objective: To determine the outcomes, demographics, clinical and surgical characteristics of NWRT in children.

Methods: Forty-three children who underwent surgery for NWRT between 1970 and 2016 were included. The charts and surgery notes were evaluated retrospectively and age, sex, complaints and physical findings at presentation, results of biochemical tests, details of surgery, pathologic findings, and the postoperative course were noted.

Results: The female to male ratio was 15:28. Distribution according to histological groups were CCSK (n=14), CMN (n=10), RCC (n=7), CN (n=4), angiomyolipoma (n=2), MRT (n=2), sclerosing nephrogenic rest (n=1), renal tubular adenoma (n=1), metanephric stromal tumor (n=1), and renal adenocarcinoma (n=1). Nephroureterectomy was the most frequently used surgical treatment (n=38) (88%). The mortality rate was 21% in malignant NWRT (3 with CCSK, 2 with MRT) and 10% in benign NWRT (2 CMN) during the five-year follow up period.

Conclusion: Non-Wilms renal tumors are rare in childhood. The presumptive diagnosis is usually a Wilms tumor. Hypercalcemia may be encountered. A nephroureterectomy is necessary in most cases. NSS can be performed in selected cases and under suitable conditions.