Primary renal sarcoma in adults. A natural history and management study by the American cancer society, Illinois division

Abstract

BACKGROUND: Primary renal sarcomas in adults are rare and unusual neoplasms. This study was performed to better define the natural history and current management of these sarcomas in a typical medical setting in the United States. METHODS: The hospital records of 4018 adult patients with renal neoplasms treated in the state of Illinois from 1975 to 1985 were examined by American Cancer Society professional volunteers. RESULTS: A primary renal sarcoma occurred in 34 patients (0.8% incidence). Eleven adult patients had Wilms tumor, 21 had primary renal sarcoma (47% leiomyosarcoma), and 2 were not found to have sarcoma on review. The median age of the patients with Wilms tumor was 30 years, whereas that of the patients with non-Wilms sarcoma was 65 years. Four of the patients with Wilms tumor (36%) are long-term survivors and all received adjuvant chemotherapy after radical nephrectomy. Six of the patients with non-Wilms sarcoma (29%) are long-term survivors after radical nephrectomy alone. CONCLUSIONS: Primary renal sarcomas, when treated with radical nephrectomy and, in the case of Wilms tumor, adjuvant chemotherapy, appear to be curable in 29-36% of cases. Histologic review of patients younger than 40 years of age with renal neoplasia is recommended.