Small Cell Carcinoma of the Female Genital Tract

Small cell carcinoma (SCC) of the female genital tract is rare, constituting less than 2% of all gynecologic malignancies. It occurs most frequently in the cervix but can also occur in the endometrium, ovary, fallopian tube, vagina, and vulva. SCC of the genital tract is microscopically indistinguishable from that of the lung. Neuroendocrine differentiation is often manifested by a histologic growth pattern, argyrophilia, ultrastructural demonstration of secretory granules, and expression of neuroendorine markers. Patients with SCC of the female genital tract may be asymptomatic but usually present with localized pain, vaginal bleeding, abdominal bloating or a mass, or symptoms of metastasis disease to the liver, bone, lung, or regional lymph nodes. Ectopic Cushing's syndrome has been reported in SCC of the vagina, and hypercalcemia and inappropriate secretion of antidiuretic hormone have been noted with SCC of the ovary. In general, these tumors have an aggressive clinical course with a propensity for extensive local invasion and distant metastases. Therapy has included surgery, radiation, and chemotherapy akin to those regimens used for SCC of the lung. Although there are no randomized clinical trials, it appears that multimodality therapy is associated with the best results and is the treatment of choice for most patients. Despite aggressive therapy, however, the prognosis for SCC of the female genital tract is poor, with only a minority of patients enjoying a prolonged survival. Indeed, the majority of patients have an early demise with extensive distant disease. We review the clinical features, evaluation, and management of SCC of the female genital tract based on a comprehensive review of the literature.