Uterine angiosarcoma is exceedingly rare, with few cases reported in literature. The prognosis is very poor, and no standardized treatment protocol has been established to date. We report here a 54-year-old postmenopausal woman with no significant medical history, including no prior uterine fibroids. She presented with severe fatigue and pallor, without other symptoms. Abdominal ultrasound revealed an abdominopelvic mass, and pelvic MRI suggested an atypical myoma. Total hysterectomy with bilateral salpingo-oophorectomy was performed. Grossly, we found in uterus a 19 cm heterogeneous, whitish-gray tumor with extensive necrosis and hemorrhage. Microscopically, the tumor was poorly differentiated, composed of epithelioid cells with focal vasoformative features. Immunohistochemical analysis showed positivity for CD31, ERG, and pancytokeratin. Clinical course was unfavorable, with multiple recurrences. She died one month after the initial diagnosis. Diagnosis of uterine angiosarcoma is challenging due to its rarity, nonspecific clinical and radiological features and broad histopathological differential diagnoses. A Correct diagnosis is critical to ensuring optimal management and care. Meticulous histopathological examination is crucial to raise suspicion of this uncommon entity and to incorporate endothelial markers into immunohistochemical panel.
Keywords: Angiosarcoma, Uterus, Sarcoma, Pathology, Diagnosis.
