Ovarian Angiosarcoma With Intractable Intraperitoneal Hemorrhage: A Case Report and Review of the Literature.

Primary ovarian angiosarcoma is an extremely rare malignant soft tissue tumor that arises from the vascular endothelium, with a limited number of reports. It has no specific symptoms, and it is rarely diagnosed in the early stages because of rapid progression. Furthermore, no standard drug therapy is available, and the prognosis is poor.  The patient was a 68-year-old woman suspected of having a ruptured ovarian tumor on plain abdominal computed tomography and anemia was observed. An emergency laparotomy was performed to stop the bleeding. Thereafter, one transcatheter arterial embolization, three laparotomies, and local radiotherapy were performed in an attempt to achieve hemostasis. However, the intraperitoneal bleeding remained intractable, and the transfusion was terminated after thorough consultation with the patient. The patient died 44 days after admission. Autopsy findings confirmed the diagnosis of primary ovarian angiosarcoma and pulmonary metastasis. Intractable intraperitoneal hemorrhage similar to that observed in this case has been reported, and because the prognosis is extremely poor, establishing an appropriate treatment regimen is desirable. Previously, the most commonly used drug regimens included anthracyclines and ifosfamide. However, recently, the possibility of treatment with molecular targeted agents and immune checkpoint inhibitors has been reported. In the future, case accumulation and genetic analyses will be required to establish appropriate treatment methods.