Primary epithelioid angiosarcoma of the adrenal gland: Report of a case and review of the literature

Abstract

Malek Mohamed Ayadi, Nadia Boujelben, Raoudha Doghri, Mouna Rezgani, Imen Abbes, Maha Driss, Riadh Chargui, Karima Mrad, Khaled Rahal

Primary mesenchymal neoplasms of the adrenal gland are extremely rare. The most common primary sarcoma is adrenal angiosarcoma. We report the case of a 51-year-old patient who presented with left flank pain. After ultrasound investigations, the patient underwent surgical removal of an adrenal tumor. The gross adrenal specimen showed extensive cystic changes with old hemorrhage and necrosis. Histologically, irregular branching vascular channels were seen intermixed with solid areas of epithelioid cells. Immunohistochemical staining of tumor cells was positive with antibodies to cytokeratin and CD31. After a 12 months follow-up, the patient is still well with no sign of a relapse. Because of the epithelioid appearance and frequent expression of epithelial immunohistochemical markers, primary epithelioid angiosarcoma of the adrenal gland can be confounded with adrenal carcinomatous metastases. We report our findings with a brief literature review and discussion of differential diagnosis.