Primary ovarian leiomyosarcomas are extremely rare tumors that comprise less than 0.1% of all ovarian malignancies. Their origin, etiology, histologic features, clinical behavior, and optimal treatment are still obscure. Malignant behavior is almost always associated with any two of coagulative necrosis, cellular atypia, and mitotic index greater than 10. Immunohistochemically, ovarian leiomyosarcomas are characterized by the expression of alpha-SMA and bcl-2. Traditionally, the International Federation of Gynecology and Obstetric (FIGO) staging and treatment of ovarian sarcomas have been the same as for epithelial ovarian carcinomas. Although surgery was performed for all cases, the extent of surgery is debatable. The benefit and modality of adjuvant therapy are controversial. The prognosis of primary ovarian leiomyosarcomas is extremely poor depending on tumor stage, tumor size, grade, and mitotic index and mostly recurs in abdomen and pelvis. We present a case of primary leiomyosarcoma of the ovary in a 48-year-old woman.
Stella et al. (Thu,) studied this question.