Aldosteronism and Precocious Puberty Due to an Ovarian Androblastoma (Sertoli Cell Tumor)¹

Abstract

This report describes a 9-year-old girl with precocious puberty and aldosteronism associated with an ovarian Sertoli cell tumor. After excision of the neoplasm there was amelioration of the manifestations of early feminization and aldosteronism. It is our impression that both endocrinopathies were induced by secretions from the ovarian tumor. Grossly both adrenals, and microscopically the simultaneously excised right adrenal, were normal. Transient hyperkalemia and absence of urinary aldosterone shortly after surgery suggested previous suppression of the remaining adrenal by an abnormal source of aldosterone. Functional ovarian neoplasms are a rare cause of early feminization. Sertoli cell tumors are uncommon; they are functional and secrete estrogens, but have not been shown to possess adrenocortical activity. Homogenates of this tumor exhibited low levels of aldosterone-forming and other adrenal steroidogenic enzymatic activities.