Primary adrenal angiosarcoma is an extremely rare vascular tumor. We report a case of a 63-year-old man with a collision tumor of epithelioid angiosarcoma and adrenocortical adenoma of the right adrenal gland. The adrenal tumor was incidentally observed by a preoperative computed tomography (CT) scan of penis squamous cell carcinoma. The patient underwent a right laparoscopic adrenalectomy, and the tumor size measured 34 × 34 × 15 mm. Histological examination revealed two different tumor cell proliferations, namely epithelioid angiosarcoma and adrenocortical adenoma. He had no symptoms or abnormality in his endocrine studies, so the adrenocortical adenoma was considered non-functioning. Three months after the adrenalectomy, bilateral pleural metastasis was observed by CT scan and pleural biopsy. Paclitaxel monotherapy was performed, and the tumor retreated. The patient died one and a half years after the adrenalectomy, but the cause of death was believed to be another disease (metastatic penis squamous cell carcinoma). To the best of our knowledge, this is the fourth report of primary adrenal angiosarcoma combined with adrenocortical adenoma.
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