Primary leiomyosarcoma of the vagina. A case report and literature review

Primary vaginal leiomyosarcoma is a rare tumor. We report a unique case of a 27-year-old woman with stage I, high-grade primary leiomyosarcoma of the vagina treated with surgical resection and adjuvant radiation therapy. She returned within 6 months with an abdominal-pelvic recurrence and lung metas tases. The patient died of disease 9 months after diagnosis. A comprehensiv e review of primary vaginal leiomyosarcoma was performed and factors affect ing survival were analyzed. A Medline search of the English-language literature revealed 66 previously reported cases. Forty-eight of these had follow-up data. Survival probabili ties were calculated using the Kaplan-Meier method, and the effects of age, stage, grade, tumor location, and treatment modality were analyzed. Stage III and IV data were combined. The overall 5-year survival rate was 43%. Patients more than 50 years of ag e had a 5-year survival rate of 26% compared with 51% for those less than 4 0 years. Five-year survival for stage I and II tumors was 55% and 44%, resp ectively. Patients with stage III/IV disease had 25% survival at 18 months. No patient treated primarily with chemotherapy or radiation therapy surviv ed beyond 36 months. In contrast, patients treated primarily with surgery h ad a 5-year survival rate of 57%. Only stage remained an independent predic tor of survival on Cox regression analysis. We continue to recommend surgical resection as primary treatment. Exenterat ion may be an option for select patients, but ultimately management should continue on a case-by-case basis.