title: Macroorchidism and panhypopituitarism: two different forms of presentation of FSH-secreting pituitary adenomas in adolescence.
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Paediatric Endocrinology Unit, Department of Paediatrics, Vall d'Hebron Hospital and Autonomous University of Barcelona, Barcelona, Spain. mclement@vhebron.net
authors.forename: María
authors.initials: M
authors.lastname: Clemente
authors.forename: Fabiola
authors.initials: F
authors.lastname: Caracseghi
authors.forename: Miquel
authors.initials: M
authors.lastname: Gussinyer
authors.forename: Diego
authors.initials: D
authors.lastname: Yeste
authors.forename: Marian
authors.initials: M
authors.lastname: Albisu
authors.forename: Elida
authors.initials: E
authors.lastname: Vázquez
authors.forename: Arantxa
authors.initials: A
authors.lastname: Ortega
authors.forename: Antonio
authors.initials: A
authors.lastname: Carrascosa
mesh_terms: D000236:Adenoma / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D000293:Adolescent; D002648:Child; D005640:Follicle Stimulating Hormone / Q000097:blood / Q000378:metabolism*; D006801:Humans; D006965:Hyperplasia / Q000209:etiology / Q000473:pathology; D007018:Hypopituitarism / Q000209:etiology*; D028322:Inhibin-beta Subunits / Q000097:blood; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D010911:Pituitary Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D013733:Testicular Diseases / Q000209:etiology* / Q000473:pathology; D013737:Testis / Q000473:pathology
chemical_list: D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D028322:Inhibin-beta Subunits