Stage I small cell carcinoma of the vulva treated with vulvectomy, lymphadenectomy, and adjuvant chemotherapy.

Neuroendocrine small cell carcinoma is a lethal, but rare, tumor that arises most frequently in the lung. Small cell cancer also rarely may occur in the female genital tract, usually in the cervix. This article concerns the fourth reported case of neuroendocrine small cell carcinoma of the vulva. Previously, small cell carcinoma of the vulva has been treated with regional therapy including surgery and radiation. Survival has been poor, however, due to the propensity of these tumors to metastasize early in the course of the disease. Recently, the median survival of patients with small cell carcinoma of the lung has been improved with the use of chemotherapy. The authors review the literature on the treatment of small cell carcinoma of the vulva and report on a patient who was treated successfully with vulvectomy and inguinal lymphadenectomy followed by adjuvant chemotherapy.