Profile and Possible Origin of an Adrenocortical Carcinoma

A 30-yr-old female with hirsutism, amenorrhea and 17-ketosteroid elevation of unknown etiology of 9-yr duration ultimately developed an adrenocortical carcinoma. The patient died 4 months after removal of the primary neoplasm of metastatic carcinoma. Studies over the 9-yr period prior to the obvious emergence of the carcinoma revealed ketosteroid suppression following glucocorticoid administration. At autopsy, the nontumorous adrenal gland was hyperplastic (20 g), suggesting that it might be functionally abnormal. These findings are consistent with an unusual adrenocortical carcinoma that was not autonomous; alternatively, a carcinoma could have evolved over time on a substrate of abnormal hyperplastic adrenal tissue.