Abstract

Malignant fibrous histiocytoma is the most common type of soft tissue sarcoma in adults. Primary malignant fibrous histiocytoma of the ovary is extremely rare, with only three previously reported cases. We reported a rare and uncommon localization of malignant fibrous histiocytoma in a 22-year-old woman. She was referred for adjuvant chemotherapy to our center with the diagnosis of storiform-pleomorphic malignant fibrous histiocytoma. A left adnexal mass was detected by computed tomography of the lower abdomen. Therefore, we decided to perform reoperation for debulking, and left salpingo-oopherectomy, total hysterectomy, infracolic omentectomy, and resection of all visible peritoneal implants were performed. Histopathologic examination revealed inflammatory, malignant fibrous histiocytoma. Immunohistochemical staining confirmed positive reactions to alpha 1-antitrypsin, vimentin, CD68, and S100. The management of malignant fibrous histiocytoma is controversial because of the heterogenous nature of the disease. Resection of all macroscopic disease is independently associated with improved disease-specific survival, and adjuvant chemotherapy for nonmyxoid variants could be acceptable alternatives if the surgical margins are tumor free.