Primary ovarian angiosarcoma: a case report and literature review.

Primary ovarian angiosarcoma is extremely rare. Only 16 cases have histologically been reported to date in the literature. A case of angiosarcoma arising in the right ovary of a 46-year-old female is presented. Grossly, the resected right ovary was completely replaced by a solid tumor mass, which revealed multiple necrotic and/or hemorrhagic foci. This case revealed the typical histological features of angiosarcoma with sinusoidal and solid patterns of anaplastic tumor cells. Immunohistochemically, tumor cells were strongly and diffusely positive for CD31 and CD34, in particular, along the cytoplasmic membrane of the tumor cells. Ultrastructurally, tumor cells possessed the intermediate junctions between tumor cells, discontinuous basal laminae attached to the irregularly shaped blood vessels and occasional cytoplasmic pinocytotic vesicles. These findings confirmed the case as being one of angiosarcoma of the ovary. The patient died 9 months after surgery as a result of developed multifocal brain metastases. A total of 17 cases reported as primary ovarian angiosarcoma, including this presented case, are clinicopathologically reviewed.