Macroorchidism and Panhypopituitarism: Two Different Forms of Presentation of FSH-Secreting Pituitary Adenomas in Adolescence

• Published in: Hormone research in paediatrics Vol. 75; no. 3; pp. 225 - 230
• Main Authors: Clemente, María, Caracseghi, Fabiola, Gussinyer, Miquel, Yeste, Diego, Albisu, Marian, Vázquez, Elida, Ortega, Arantxa, Carrascosa, Antonio
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01.01.2011
• Subjects: Adenoma - blood; Adenoma - diagnosis; Adenoma - pathology; Adenoma - physiopathology; Adolescent; Child; Follicle Stimulating Hormone - blood; Follicle Stimulating Hormone - secretion; Humans; Hyperplasia - etiology; Hyperplasia - pathology; Hypopituitarism - etiology; Inhibin-beta Subunits - blood; Luteinizing Hormone - blood; Male; Novel Insights from Clinical Practice; Pituitary Neoplasms - blood; Pituitary Neoplasms - diagnosis; Pituitary Neoplasms - pathology; Pituitary Neoplasms - physiopathology; Testicular Diseases - etiology; Testicular Diseases - pathology; Testis - pathology; FSH pituitary adenoma; Gonadotroph adenoma; Inhibin B
• ISSN: 1663-2818; 1663-2826
• DOI: 10.1159/000322211

Background: FSH-secreting pituitary adenomas are extremely rare in children and are seldom associated with clinical manifestations of high serum gonadotrophin levels. Thus, most patients have a late presentation, usually as macroadenomas. Case Reports: Two different clinical forms of presentation of FSH-secreting pituitary adenomas are reported: one in a 12-year-old boy with macroorchidism due to a pituitary microadenoma, probably FSH-secreting, and the other in a 15-year-old boy with panhypopituitarism due to an FSH-producing macroadenoma. Both patients presented slightly high or high FSH with low LH and high inhibin B levels. In the first case, the microadenoma was treated medically with cabergoline, which failed to reduce FSH and inhibin B levels. No radiological progression has been observed despite increasing testicular volume. In the second case, surgery was performed on the macroadenoma, leading to a decrease in FSH and inhibin B levels. The patient developed severe hypothalamic obesity and is currently under treatment with somatostatin. Conclusions: FSH-secreting pituitary tumors have an extremely variable clinical expression. The discrepancy between normal or slightly increased FSH and low LH values, together with high inhibin B levels, strongly suggests FSH hypersecretion which should be studied.