Primary ovarian angiosarcoma is extremely rare. Only 16 cases have histolog
ically been reported to date in the literature. A case of angiosarcoma aris
ing in the right ovary of a 48-year-old female Is presented. Grossly, the r
esected right ovary was completely replaced by a solid tumor mass, which re
vealed multiple necrotic and/or hemorrhagic foci. This case revealed the ty
pical histological features of angiosarcoma with sinusoidal and solid patte
rns of anaplastic tumor cells. Immunohistochemically, tumor cells were stro
ngly and diffusely positive for CD31 and CD34, in particular, along the cyt
oplasmic membrane of the tumor cells. Ultrastructurally, tumor cells posses
sed the intermediate junctions between tumor cells, discontinuous basal lam
inae attached to the irregularly shaped blood vessels and occasional cytopl
asmic 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 report
ed as primary ovarian angiosarcoma, including this presented case, are clin
icopathologically reviewed.