Primary pure ovarian leiomyosarcomas constitute a malignant subgroup of ovarian smooth muscle tumors which comprise only 1% of ovarian tumors. Their origin, etiology, histologic features, clinical behavior, and optimal treatment are still obscure. Malignant behavior is almost always associated with any 2 of coagulative necrosis, cellular atypia, and mitotic index greater than 10. Immunohistochemical and electron microscopic evaluations may improve diagnostic accuracy. Traditionally, International Federation of Gynecology and Obstetrics (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. Benefit and modality of adjuvant therapy is controversial. The prognosis of primary pure ovarian leiomyosarcomas is extremely poor depending on tumor stage, tumor size, grade, and mitotic index and mostly recurs in abdomen and pelvis.
Target audience: Obstetricians & Gynecologists, Family Physicians.
Learning objectives: After completion of this article, the reader should be able to state how rare primary ovarian leiomyosarcoma (POLMS) is, explain that because of its rarity the best diagnostic and treatment modalities are not conclusive, and recall that the authors reviewed the literature to bring the readership current on POLMS.