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

BACKGROUND

Sarcomas of the female genital tract are very rare. A small subset of these, primary ovarian angiosarcoma, is even more rare, with only 26 cases reported in the literature. Most cases of advanced-stage disease are aggressive and demonstrate a poor response to both surgery and chemotherapy with an overall poor prognosis.

CASE: We present a case of a previously healthy 67-year-old woman who presented with abdominal pain and profound anemia. She underwent a full staging and optimal debulking that revealed Stage IIIC primary ovarian angiosarcoma. The patient died 3 weeks post-operatively secondary to hemorrhage from progressive, metastatic tumor sites prior to initiation of chemotherapy.

CONCLUSION

Primary ovarian angiosarcoma is extremely rare, often presenting in later stages with nonspecific symptoms. Immunohistochemical staining of pathological specimens can aid in the diagnosis. The optimal regimen for adjuvant chemotherapy is unknown, and the prognosis is very poor.