Primary Ovarian Epithelioid Angiosarcoma with Extensive Lymph Node Metastases: A Case Report and Literature Review

Primary ovarian epithelioid angiosarcoma (EAS) is an exceptionally rare and highly aggressive vascular malignancy with only isolated cases reported in the literature. Owing to its nonspecific clinical presentation and overlapping radiological features, preoperative diagnosis is extremely challenging and often mimics more common gynecologic malignancies. Consequently, evidence regarding optimal diagnosis and management remains limited. A 61-year-old postmenopausal woman presented with abdominal pain and a rapidly enlarging pelvic mass. Ultrasonography, computed tomography, and magnetic resonance imaging demonstrated a giant hypervascular pelvic tumor with extensive pelvic and para-aortic lymphadenopathy, initially suggesting uterine sarcoma. Serum CA125 and lactate dehydrogenase levels were elevated. Exploratory laparotomy identified a dominant right ovarian hypervascular tumor with diffuse peritoneal dissemination and bulky pelvic and para-aortic nodal metastases. Comprehensive clinical, radiological, intraoperative, gross pathological, histopathological, and immunohistochemical assessment demonstrated a primary ovarian origin, with no evidence of an extra-ovarian primary angiosarcoma. The patient underwent radical cytoreductive surgery, including total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendectomy, resection of visible metastatic lesions, and systematic pelvic and para-aortic lymphadenectomy, achieving complete macroscopic cytoreduction. Histopathological examination demonstrated epithelioid tumor cells with vasoformative differentiation, while immunohistochemistry showed diffuse strong positivity for CD31, CD34, and FLI1, confirming primary ovarian epithelioid angiosarcoma. The patient declined adjuvant chemotherapy, developed rapid postoperative disease progression, and died approximately 2 months after surgery. This case highlights the marked diagnostic challenges and highly aggressive biological behavior of primary ovarian EAS with extensive pelvic and para-aortic lymph node metastases and diffuse intra-abdominal dissemination. Accurate diagnosis required a comprehensive clinicopathological correlation supported by histopathological and immunohistochemical evaluation. Although complete macroscopic cytoreduction and systematic lymphadenectomy were technically feasible, their therapeutic benefit cannot be inferred from a single case. This report expands the limited literature and emphasizes the need for improved diagnostic strategies, collaborative data collection, and more effective systemic therapies for this rare malignancy.