Adrenal angiosarcoma with metastasis: Imaging and histopathology of a rare adrenal cancer

Adrenal angiosarcoma is an extremely rare malignancy with few reported cases in the literature. Patients may be asymptomatic or have nonspecific complaints at presentation. There are no pathognomonic imaging findings, while histopathology can be confounding due to overlap with other disease processes. We present a case of a 38-year-old woman with a long history of cocaine abuse who had metastatic adrenal angiosarcoma at the time of presentation. The adrenal tumor was an incidental finding on imaging. CT demonstrated a heterogeneous mass in the right adrenal gland with central calcification, and MRI identified central necrosis in the mass. Histopathology demonstrated sheets of epithelioid cells, dilated anastomotic vascular spaces, and abundant necrosis, and immunohistochemistry was positive for various vascular markers. The findings were consistent with adrenal angiosarcoma. The patient underwent adrenalectomy and is now receiving adjuvant chemotherapy. Due to the aggressive nature of adrenal angiosarcoma, timely diagnosis and treatment is critical. This case adds to the sparse literature surrounding this disease by highlighting crucial imaging and histopathologic findings that will aid in more efficient diagnosis. Although rare, the disease should be considered in the context of suspicious adrenal lesions. In the future, structured review of all reported cases of adrenal angiosarcoma can help inform diagnosis and therapy for this rare disease.