Ovarian angiosarcoma: Extended survival following optimal cytoreductive surgery and adjuvant chemotherapy

Angiosarcomas are rare vascular tumors that comprise up to 3% of all adult soft tissue sarcomas. The majority of angiosarcomas occur in cutaneous sites, most frequently in the head and neck region. They can be caused by prior radiation, chronic lymphedema, or a history of exposure to vinyl-chloride chemotherapy (Bradford et al., 2010). Although one-fourth of these tumors arise in deep soft tissue, angiosarcoma has only rarely been described in the ovary. The first reported case was in 1931 and, to date, less than 35 cases have been published in the literature (Bosmuller et al., 2011). Angiosarcomas, regardless of anatomic site, demonstrate aggressive clinical behavior, with median survival ranging from 15 to 30 months, and fewer than 12% of patients alive at 5 years (Penel et al., 2007). In general, patients with ovarian angiosarcoma present with advanced disease and have a very poor prognosis. Among those patients with FIGO stage III and IV disease, the median survival is 6 to 7 months, with an overall range of 1 to 30 months (Bradford et al., 2010). Typically, radical surgical excision is followed by any number of possible chemotherapeutic regimens. For metastatic soft-tissue sarcomas, anthracyclines and ifosfamide have proven to be the most effective chemotherapeutic agents. Additionally, taxanes have demonstrated promising results in angiosarcomas of the head and neck (Penel et al., 2008). We report a patient with stage IIIC ovarian angiosarcoma who had extended survival after primary cytoreductive surgery and adjuvant doxorubicin and ifosfamide chemotherapy.