Primary ovarian angiosarcoma presenting as malignant cells in ascites: Case report and review of the literature

Abstract

Primary angiosarcoma of the ovary is a rare tumor, with less than 25 cases reported in the literature. These tumors are thought to arise from carcinosarcomas, teratomas or the ovarian vasculature, and occur at any age. The prognosis is dismal in the majority of cases. We present the case of a 19-year old female who presented with abdominal distention and abdominal pain. Tapping of peritoneal effusion showed groups of pleomorphic cells that were negative for epithelial and germ cell markers. Immunohistochemistry performed on the surgical specimen of the ovary, and subsequently on the effusion specimen showed staining for endothelial markers. The patient was treated post-operatively with chemotherapy, but died one year following diagnosis. This is the first reported case of an ovarian angiosarcoma that metastasized to the peritoneal cavity, with a resulting malignant effusion. Despite the rarity of metastasis from gynecological sarcomas in effusions, this possibility needs to be included in the differential diagnosis of malignant effusions that are negative for epithelial and germ cell markers.