Therapy and outcome of small cell carcinoma of the kidney - Report of two cases and a systematic review of the literature

BACKGROUND. Primary small cell carcinoma originating from the kidneys is an extremely rare neoplasm. The authors described two patients with small cell carcinoma of the kidney and provided a systematic review of the literature to detail the clinical characteristics and therapy of this rare tumor. METHODS. MEDLINE and CANCERLIT literature search was performed from 1966 to 2002 for articles on small cell carcinoma of the kidney. Twenty-two patients with small cell carcinoma of the kidney and renal pelvis were reviewed. RESULTS. The median age at diagnosis was 62 years and there was a female preponderance (male:female ratio, 1:3.4). Abdominal pain (70%) was the most commonly reported symptom. Distant metastases were present in 32% of patients at the time of diagnosis. Surgery and systemic chemotherapy were the primary therapeutic modalities utilized (nephrectomy alone: 9 patients; nephrectomy and chemotherapy: 10 patients; chemotherapy alone: 3 patients). The median survival was 8 months (range, < 1-101 months). The use of platinum-based chemotherapy was predictive of an improved overall survival (median survival was 20 months in patients receiving a platinum-containing regimen compared with 8 months in those who did not receive platinum; P = 0.02). CONCLUSIONS. Small cell carcinoma of the kidney is an extremely rare neoplasm that resembles its counterparts arising from the tracheo-bronchial and other extrapulmonary sites in its aggressive behavior and high propensity for locoregional and distant dissemination. Clinical presentation is usually late in the course of the disease. The use of platinum-based chemotherapy has been associated with tumor regression and prolonged survival. (C) 2003 American Cancer Society.