Choosing the best management of uterine and vulvo-vaginal sarcomas depends on careful histologic review of the pathologic specimen. Prognosis and treatment vary greatly depending on specific histology, grade, and tumor stage. The initial approach to sarcomas of the female genital tract, with the occasional exception of vulvo-vaginal rhabdomyosarcoma, is surgery. Adjuvant radiation decreases local recurrence rates for uterine sarcomas, but has not been clearly shown to improve overall survival. It is frequently used as adjuvant therapy for resected high-grade or margin-positive vulvo-vaginal sarcomas, and for endometrial stromal sarcomas. Adjuvant chemotherapy has not been demonstrated to improve survival in vulvo-vaginal sarcomas, with the exception of vulvo-vaginal rhabdomyosarcomas, nor has it been demonstrated to improve survival in uterine sarcomas. Chemotherapy may be used for recurrent or persistent disease. The choice of agent depends on the histologic type of sarcoma.
