Primary angiosarcoma of the ovary: a case report and review of the literature.

INTRODUCTION: Gynecological sarcomas are rare and have a poor prognosis. Uterine sarcomas are most common accounting for 4% of all uterine tumors. Ovarian sarcomas are less frequent and are usually carcinosarcomas. CASE REPORT: A previously healthy 40-year-old G2P2 presented for evaluation of 72 h of right upper quadrant pain and shortness of breath. A malignant right pleural effusion, ascites, and adnexal mass were found. Surgical staging and suboptimal debulking revealed pure angiosarcoma of the ovary Stage IV. DISCUSSION: There are 12 cases of ovarian angiosarcoma reported in the literature. Ten of these cases presented in advanced stages with survivals of 2-30 months. Various chemotherapy regimens have been tried on these tumors including the most recent recommendation of MAID (mesna, doxorubicin, ifosfamide, and dacarbazine) and prognosis remains poor. Our patient underwent elective right pleurodesis via video-assisted thorascopic surgery under local anesthesia for an early recurrent right pleural effusion and subsequently began MAID chemotherapy. Copyright 1999 Academic Press.