Isosexual Precocity in Boys Including a Case of a Gonadotropin-Producing Teratoma

Abstract

Our report presents the etiologic classification of male isosexual precocity and the distribution of causes in 21 boys with this condition seen at The New York Hospital-Cornell Medical Center between 1932 and 1955. One patient, W.P., with precocious puberty due to a hormonogenic teratoma producing pituitary or pituitary-like gonadotropins (FSH) is, to our knowledge, the first such case to be reported. The differential diagnosis of isosexual precocity in boys is relatively simple and rests on careful clinical examinations and a few laboratory studies (24). In addition to the macrogenitosomia, which was present in all of the boys, the important clinical features include the age of onset, associated systemic and neurologic symptoms, and the size of the testes. The essential laboratory tests include urinary assays for 17-ketosteroids and pituitary gonadotropins, testicular biopsies, roentgenograms of the skull, abdomen, and skeleton, and intravenous pyelography. Only rarely are more elaborate laboratory studies needed for differential diagnosis. The most important therapeutic test, which applies only to the differentiation between adrenocortical hyperplasia and tumor, is the prompt and continued suppression of 17-ketosteroid excretion by the administration of cortisone or hydrocortisone. Finally, in all conditions associated with sexual precocity, there is an even greater need than usual for the physician to provide supportive psychologic help to the children and their families.