Primary adrenal gland epithelioid sarcoma: A case report and literature review

Epithelioid sarcoma is a malignant mesenchymal neoplasm with morphologic and immunophenotypic epithelioid differentiation, which rarely arises in solid organs. We report a case of primary epithelioid sarcoma in the adrenal gland of a 31-year-old female. The patient initially presented with nausea and rectal bleeding, and subsequent imaging studies revealed a 4.4 cm left adrenal gland mass and left retroperitoneal lymphadenopathy. Clinical and radiological studies did not reveal tumor elsewhere in the patient. Histologic features were those of epithelioid sarcoma, proximal type with cohesive clusters of epithelioid tumor cells harboring frequent mitoses, and areas of necrosis. Immunohistochemical stains showed strong, diffuse expression of epithelial markers (pancytokeratin), and CD34 and Fli-1. Partial and focal positive staining of CK7 was also noted. Nuclear expression of SMARCB1 (INI-1) protein was lost. ERG was negative in this case. We believe that this is the second-case report of a primary adrenal gland epithelioid sarcoma. Fli-1 positivity was seen in our case, and ERG was negative as shown in some recent publications regarding epithelioid sarcomas.