Primary adrenal epithelioid angiosarcoma: An extraordinarily rare malignancy with a challenging diagnosis

Adrenal epithelioid angiosarcoma is an exceedingly rare vascular endothelial neoplasm, accounting for a minute fraction of sarcomas with fewer than 60 cases reported in the medical literature. We report the case of a 70-year-old female with primary adrenal epithelioid angiosarcoma, presenting with a rapidly enlarging right adrenal mass and associated severe right upper quadrant pain. Biochemical testing revealed that the mass was nonfunctional, and contrast-enhanced computed tomography revealed a large, heterogeneously enhancing lesion with inferior vena cava compression. Given rapid interval growth and severe symptoms, she underwent laparoscopic adrenalectomy, and histopathology with immunohistochemistry confirmed a diagnosis of primary adrenal epithelioid angiosarcoma. Postoperative management included paclitaxel chemotherapy, and the patient has remained disease-free for 2 years post-operation. This case emphasizes that the clinical and imaging features of adrenal epithelioid angiosarcoma are nonspecific, and definitive diagnosis requires surgical pathology with vascular marker staining to avoid misclassification as metastatic carcinoma. Timely resection in combination with chemotherapy can yield favorable outcomes despite this tumor’s aggressive behavior and overall poor prognosis.