Primary pure angiosarcoma of the ovary: A case report

Ovarian angiosarcomas are rare tumors, with an incidence of one in 1,000,000 malignant ovarian tumors, which may present in a background of carcinosarcoma or teratoma. However, primary pure ovarian angiosarcoma is even rarer with a few cases published in the literature. Here, we present a case of a perimenopausal woman who presented with a 16 cm adnexal mass. She underwent bilateral salpingo-oophorectomy with omentectomy, which on histopathological examination revealed an ovarian angiosarcoma with tumor deposits in the pelvic peritoneum. No other component was demonstrable within the tumor, in light of which it was reported as a primary pure ovarian angiosarcoma. She received adjuvant paclitaxel- and carboplatin-based chemotherapy after discussion in a multispecialty tumor board. This case highlights the gross and microscopic findings of primary ovarian angiosarcomas and their distinction from their differential diagnosis.