The sarcomatoid variant of renal cell carcinoma is a clinical entity with local and metastatic aggressiveness which translates to short survival time. Fourteen cases of patients that fit strict criteria for sarcomatoid cell carcinoma and convenient clinical data for treatment and follow-up were retrospectively selected among 1,235 patients treated at the Institut Gustave-Roussy over a 17-year period. Thirteen patients underwent initial nephrectomy. Local recurrence or distant metastases occurred in all patients. Only 1 patient was treated by surgical removal of a unique lung metastasis and is currently alive with no evidence of disease. No response was observed in all 4 patients treated with alpha-interferon. Chemotherapy was administered in 10 patients, 8 of whom received doxorubicin-based regimens. The survival was 20, 29 and 60 months for the 3 responding patients as compared to a median of 9 months for patients with progressive disease. Further phase II studies are warranted to define the real impact of chemotherapy in this disease.