Abstract
Angiosarcoma is an extremely uncommon mesenchymal neoplasm overall and moreso in female genital organs such as the ovary. Diagnosing primary ovarian angiosarcoma remains challenging on clinical grounds due to the absence of specific clinical symptoms as well as on histopathological analysis especially in poorly differentiated subtypes due to non-specific and overlapping morphologic features. Misdiagnosis in such scenarios can be devastating as this tumor is clinically very aggressive. We describe a case of primary ovarian angiosarcoma in a 33-year-old multiparous female with bilateral ovarian masses and metastasis at diagnosis. Histopathologic appraisal revealed a poorly differentiated malignant tumor with varied differential diagnoses. The saviour in such a scenario was the immunohistochemistry findings, underlining the incredible utility of this technique in the precise diagnosis and evasion of misdiagnosis. This case accentuates the paramount importance of precise diagnostic modalities in shaping clinical practice and enriching the scientific understanding of rare and aggressive neoplasms. Against this backdrop, the potential pitfalls and pearls while dealing with this entity have been elucidated, along with a review of the recent literature.