Macroorchidism and panhypopituitarism: two different forms of presentation of FSH-secreting pituitary adenomas in adolescence

Horm Res Paediatr. 2011;75(3):225-30. doi: 10.1159/000322211. Epub 2010 Dec 23.


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.

Publication types

  • Case Reports

MeSH terms

  • Adenoma / blood
  • Adenoma / diagnosis*
  • Adenoma / pathology
  • Adenoma / physiopathology
  • Adolescent
  • Child
  • Follicle Stimulating Hormone / blood
  • Follicle Stimulating Hormone / metabolism*
  • Humans
  • Hyperplasia / etiology
  • Hyperplasia / pathology
  • Hypopituitarism / etiology*
  • Inhibin-beta Subunits / blood
  • Luteinizing Hormone / blood
  • Male
  • Pituitary Neoplasms / blood
  • Pituitary Neoplasms / diagnosis*
  • Pituitary Neoplasms / pathology
  • Pituitary Neoplasms / physiopathology
  • Testicular Diseases / etiology*
  • Testicular Diseases / pathology
  • Testis / pathology


  • Luteinizing Hormone
  • Follicle Stimulating Hormone
  • Inhibin-beta Subunits