Primary adrenal angiosarcomas are exceedingly rare with a rapidly progressive clinical course and a poor outcome. Establishing the diagnosis can be challenging, and it is complicated by the fact that there are no characteristic clinical or imaging features that are pathognomonic for angiosarcoma. Histologically, they can overlap with other more commonly encountered adrenal tumors. Herein, we present an otherwise healthy 41-year-old woman diagnosed with a primary adrenal epithelioid angiosarcoma. We aim to expand the knowledge of the sparse literature existing on primary adrenal angiosarcomas to help better understand the diagnostic features, clinical behavior, and management of these rare tumors.
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