Primary sarcoma of the ovary: report of five cases and review of the literature

Primary ovarian sarcomas are rare and usually behave very aggressively. Over a ten year period (1987-1996) five cases of primary ovarian sarcoma were managed at the Soroka Medical Center, Beer-Sheva, Israel. Four patients had malignant mullerian-mixed mesodermal tumor (MMMMT): two had Stage IIIC tumor with chondrosarcoma being the predominant sarcomatous element, one had Stage IIIC tumor with high-grade endometrioid stromal sarcoma (ESS) being the predominant sarcomatous element and one had Stage IC tumor with rhabdomyosarcoma being the predominant sarcomatous element. One patient had Stage LA leiomyosarcoma (LMS). All four patients with MMMMT received postoperative adjuvant chemotherapy, whereas the patient with LMS did not. The four patients with MMMMT died of their disease 10, 10, 13 and 25 months, respectively, after initial surgery. The patient with LMS died of intercurrent disease 21 months after initial surgery. It is concluded that most patients with primary ovarian sarcoma present with extraovarian disease and the prognosis is poor. The mainstay of treatment is debulking surgery consisting of total abdominal hysterectomy, bilateral salpingo-oophorectomy and extirpation of tumor masses. The benefit of postoperative adjuvant chemotherapy and/or radiotherapy is still a subject of debate and has yet not been established.