Abstract

Myxoid variant of adrenal cortical carcinoma is extremely rare and there have been only 16 such cases reported in the medical literature. Here we report on a case of 43-year-old woman with a left adrenal mass that was detected during the evaluation for Cushing's syndrome. Left adrenalectomy was performed and the tumor weighed 347 g. The cut surface was predominantly myxoid and gelatinous with central hemorrhage and necrosis. Histologically, the tumor cells were rather small, uniform and polygonal with mild pleomorphism. It showed diverse morphologic patterns according to the amount of the myxoid stromal component. Making the diagnosis was not easy because the tumor was without areas of conventional adrenocortical carcinoma. Immunohistochemically, the tumor cells were positive for alpha-inhibin, synaptophysin and vimentin, but the tumor cells were negative for pan-cytokeratin and CAM 5.2. The immunophenotypes were identical to those of conventional adrenal cortical neoplasms. During the evaluation of a cytokeratin-negative and vimentin-positive retroperitoneal neoplasm with a myxoid component, the possibility of adrenal cortical tumor should be considered in spite that this is a very rare entity.

• Keywords: Adrenocortical carcinoma;Myxoid;Cushing syndrome