Epithelioid angiosarcoma (EAS) is a rare and highly aggressive malignant vascular neoplasm of mesenchymal origin with endothelial differentiation, accounting for approximately 1%–2% of all soft tissue sarcomas1. Epithelioid angiosarcoma is a distinct histological variant of angiosarcoma characterized by malignant endothelial cells with epithelioid morphology, marked cytologic atypia, and vasoformative differentiation, as recognized in the current World Health Organization (WHO) classification of soft tissue and bone tumors2. Epithelioid angiosarcoma has subsequently been reported in multiple anatomical locations, including the skin, breast, kidney, gastrointestinal tract, lung, and pleura3,4,5,6. Rare presentations have also been described in unusual sites such as the aorta following endovascular repair and the cranial region, where the tumor may manifest as a subdural hematoma secondary to skull involvement7,8.
Angiosarcoma is characterized by aggressive biological behavior, rapid progression, early metastatic dissemination, and poor survival outcomes. Surgical resection remains the cornerstone of treatment for localized and potentially resectable disease because it provides both diagnostic tissue and therapeutic cytoreduction, while histopathological and immunohistochemical evaluation remain essential for definitive diagnosis9,10. Complete macroscopic cytoreduction may be considered in selected patients with resectable advanced disease to achieve maximal tumor debulking and obtain adequate tissue for definitive pathological diagnosis; however, evidence supporting the therapeutic benefit of extensive cytoreduction or systematic lymphadenectomy in primary ovarian epithelioid angiosarcoma remains extremely limited because of the rarity of the disease11,12,13.
Primary ovarian angiosarcoma is an exceptionally uncommon malignancy, with only isolated cases reported in the literature9,10. The epithelioid subtype is particularly rare and is associated with an especially aggressive clinical course. Reported cases have demonstrated heterogeneous presentations, variable treatment strategies, and consistently poor outcomes11,12,13,14,15,16,17,18,19,20,21,22. Because clinical manifestations and imaging findings are nonspecific, ovarian EAS is frequently misdiagnosed preoperatively as epithelial ovarian carcinoma, metastatic disease, or uterine sarcoma9,10,19,22. In addition, metastatic angiosarcoma involving the ovary should be considered in the differential diagnosis, making comprehensive clinicoradiological, intraoperative, histopathological, and immunohistochemical correlation essential for establishing a primary ovarian origin. This diagnostic uncertainty complicates surgical planning and intraoperative decision-making, particularly regarding the extent of cytoreductive surgery and the management of clinically suspicious lymph nodes in advanced disease.
The overall goal of the present report is to expand the limited clinicopathological evidence regarding primary ovarian EAS and to provide additional insight into its diagnostic challenges, metastatic behavior, surgical management, and clinical outcomes. We present a rare case of primary ovarian epithelioid angiosarcoma with extensive pelvic and para-aortic lymph node metastases, initially misdiagnosed as uterine sarcoma, together with a review of previously published cases. The diagnosis of primary ovarian epithelioid angiosarcoma was supported by the presence of a dominant right ovarian mass, the gross and microscopic relationship of the tumor to ovarian tissue, the distribution of metastatic disease, and comprehensive clinical and radiological evaluation demonstrating no evidence of a primary angiosarcoma at another anatomical site. The rationale for presenting this case lies in the extremely rare occurrence of widespread nodal metastases and diffuse intra-abdominal dissemination, features that are scarcely documented in the current literature. Although complete macroscopic cytoreduction with systematic pelvic and para-aortic lymphadenectomy was technically feasible in this patient, the present report does not imply a therapeutic or survival benefit of this approach; rather, it illustrates its role in achieving complete gross resection, accurate pathological assessment, and documentation of the extent of disease. By contextualizing this case within the wider body of published literature, we aim to assist clinicians and surgeons in recognizing clinical scenarios in which ovarian EAS should be considered in the differential diagnosis of rapidly enlarging hypervascular pelvic tumors with extensive nodal involvement.
Case Presentation
A 61-year-old postmenopausal woman presented to the Department of Gynecology at Gansu Provincial Maternal and Child Health Hospital in March 2023 with a 2-week history of intermittent abdominal pain and progressive abdominal enlargement. Physical examination revealed a large abdominopelvic mass extending approximately 10 cm above the umbilicus. Laboratory investigations demonstrated elevated serum CA125 (156.1 U/mL) and lactate dehydrogenase (257 U/L) levels, while other routinely assessed gynecologic tumor markers were within normal limits.
Pelvic ultrasonography, contrast-enhanced computed tomography, and magnetic resonance imaging demonstrated a large hypervascular right adnexal mass with extensive pelvic and para-aortic lymphadenopathy, raising suspicion for an advanced gynecologic malignancy, initially considered to represent uterine sarcoma. Comprehensive preoperative imaging demonstrated no evidence of a primary vascular malignancy outside the pelvis.
The patient underwent exploratory laparotomy with the goal of complete macroscopic cytoreduction and definitive diagnosis. Intraoperatively, a dominant right ovarian hypervascular tumor with diffuse peritoneal dissemination and bulky pelvic and para-aortic lymph node involvement was identified, and radical cytoreductive surgery was performed. The final diagnosis of primary ovarian epithelioid angiosarcoma was established by histopathological and immunohistochemical evaluation. Despite an initially uncomplicated postoperative recovery, the patient declined adjuvant chemotherapy, developed rapidly progressive metastatic disease, and died approximately 2 months after surgery. The overall clinical course is summarized in Table 1.
Diagnosis, Assessment, and Plan
The patient presented with a rapidly enlarging abdominopelvic mass, abdominal pain, elevated serum CA125 and lactate dehydrogenase levels, and imaging findings suggestive of an advanced gynecologic malignancy. Pelvic ultrasonography, contrast-enhanced computed tomography, and magnetic resonance imaging demonstrated a large hypervascular right adnexal mass with extensive pelvic and para-aortic lymphadenopathy. Comprehensive preoperative imaging demonstrated no evidence of a primary vascular malignancy outside the pelvis.
The differential diagnosis included uterine sarcoma, epithelial ovarian carcinoma, metastatic carcinoma, and other primary gynecologic sarcomas, as well as less common vascular and epithelioid neoplasms. Because the radiological findings were nonspecific, a definitive preoperative diagnosis could not be established. Given the extensive but potentially resectable disease, exploratory laparotomy was undertaken with the goals of complete macroscopic cytoreduction and definitive pathological diagnosis. Intraoperative frozen section examination was not performed because the operative findings indicated that immediate cytoreductive surgery would be required regardless of the pathological impression.
The patient underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendectomy, resection of visible metastatic lesions, and systematic pelvic and para-aortic lymphadenectomy, achieving complete macroscopic cytoreduction. Histopathological and immunohistochemical evaluation confirmed the diagnosis of primary ovarian epithelioid angiosarcoma. Adjuvant chemotherapy was recommended because of the aggressive nature of the disease and extensive metastatic involvement; however, the patient declined further treatment and subsequently developed rapidly progressive disease.