Macroorchidism and panhypopituitarism: two different forms of presentation of FSH-secreting pituitary adenomas in adolescence. 2011

María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
Paediatric Endocrinology Unit, Department of Paediatrics, Vall d'Hebron Hospital and Autonomous University of Barcelona, Barcelona, Spain. mclement@vhebron.net

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.

UI MeSH Term Description Entries
D006965 Hyperplasia An increase in the number of cells in a tissue or organ without tumor formation. It differs from HYPERTROPHY, which is an increase in bulk without an increase in the number of cells. Hyperplasias
D007018 Hypopituitarism Diminution or cessation of secretion of one or more hormones from the anterior pituitary gland (including LH; FOLLICLE STIMULATING HORMONE; SOMATOTROPIN; and CORTICOTROPIN). This may result from surgical or radiation ablation, non-secretory PITUITARY NEOPLASMS, metastatic tumors, infarction, PITUITARY APOPLEXY, infiltrative or granulomatous processes, and other conditions. Adenohypophyseal Hyposecretion,Anterior Pituitary Hyposecretion Syndrome,Sheehan Syndrome,Simmonds Disease,Hyposecretion Syndrome, Anterior Pituitary,Hyposecretion, Adenohypophyseal,Pituitary Insufficiency,Postpartum Hypopituitarism,Postpartum Panhypopituitarism,Postpartum Pituitary Insufficiency,Sheehan's Syndrome,Simmonds' Disease,Disease, Simmonds,Hypopituitarism, Postpartum,Insufficiency, Pituitary,Panhypopituitarism, Postpartum,Pituitary Insufficiency, Postpartum,Sheehans Syndrome,Simmond's Disease,Syndrome, Sheehan,Syndrome, Sheehan's
D007986 Luteinizing Hormone A major gonadotropin secreted by the adenohypophysis (PITUITARY GLAND, ANTERIOR). Luteinizing hormone regulates steroid production by the interstitial cells of the TESTIS and the OVARY. The preovulatory LUTEINIZING HORMONE surge in females induces OVULATION, and subsequent LUTEINIZATION of the follicle. LUTEINIZING HORMONE consists of two noncovalently linked subunits, alpha and beta. Within a species, the alpha subunit is common in the three pituitary glycoprotein hormones (TSH, LH and FSH), but the beta subunit is unique and confers its biological specificity. ICSH (Interstitial Cell Stimulating Hormone),Interstitial Cell-Stimulating Hormone,LH (Luteinizing Hormone),Lutropin,Luteoziman,Luteozyman,Hormone, Interstitial Cell-Stimulating,Hormone, Luteinizing,Interstitial Cell Stimulating Hormone
D008297 Male Males
D010911 Pituitary Neoplasms Neoplasms which arise from or metastasize to the PITUITARY GLAND. The majority of pituitary neoplasms are adenomas, which are divided into non-secreting and secreting forms. Hormone producing forms are further classified by the type of hormone they secrete. Pituitary adenomas may also be characterized by their staining properties (see ADENOMA, BASOPHIL; ADENOMA, ACIDOPHIL; and ADENOMA, CHROMOPHOBE). Pituitary tumors may compress adjacent structures, including the HYPOTHALAMUS, several CRANIAL NERVES, and the OPTIC CHIASM. Chiasmal compression may result in bitemporal HEMIANOPSIA. Pituitary Cancer,Cancer of Pituitary,Cancer of the Pituitary,Pituitary Adenoma,Pituitary Carcinoma,Pituitary Tumors,Adenoma, Pituitary,Adenomas, Pituitary,Cancer, Pituitary,Cancers, Pituitary,Carcinoma, Pituitary,Carcinomas, Pituitary,Neoplasm, Pituitary,Neoplasms, Pituitary,Pituitary Adenomas,Pituitary Cancers,Pituitary Carcinomas,Pituitary Neoplasm,Pituitary Tumor,Tumor, Pituitary,Tumors, Pituitary
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005640 Follicle Stimulating Hormone A major gonadotropin secreted by the adenohypophysis (PITUITARY GLAND, ANTERIOR). Follicle-stimulating hormone stimulates GAMETOGENESIS and the supporting cells such as the ovarian GRANULOSA CELLS, the testicular SERTOLI CELLS, and LEYDIG CELLS. FSH consists of two noncovalently linked subunits, alpha and beta. Within a species, the alpha subunit is common in the three pituitary glycoprotein hormones (TSH, LH, and FSH), but the beta subunit is unique and confers its biological specificity. FSH (Follicle Stimulating Hormone),Follicle-Stimulating Hormone,Follitropin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000236 Adenoma A benign epithelial tumor with a glandular organization. Adenoma, Basal Cell,Adenoma, Follicular,Adenoma, Microcystic,Adenoma, Monomorphic,Adenoma, Papillary,Adenoma, Trabecular,Adenomas,Adenomas, Basal Cell,Adenomas, Follicular,Adenomas, Microcystic,Adenomas, Monomorphic,Adenomas, Papillary,Adenomas, Trabecular,Basal Cell Adenoma,Basal Cell Adenomas,Follicular Adenoma,Follicular Adenomas,Microcystic Adenoma,Microcystic Adenomas,Monomorphic Adenoma,Monomorphic Adenomas,Papillary Adenoma,Papillary Adenomas,Trabecular Adenoma,Trabecular Adenomas
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths

Related Publications

María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
September 1976, The Journal of clinical endocrinology and metabolism,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
January 1980, Annales d'endocrinologie,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
June 2002, Surgical neurology,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
October 1979, Acta neuropathologica,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
October 1980, La Revue du praticien,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
January 1994, Clinical endocrinology,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
January 1981, International advances in surgical oncology,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
April 1995, Bailliere's clinical endocrinology and metabolism,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
August 1996, Metabolism: clinical and experimental,
María Clemente, and Fabiola Caracseghi, and Miquel Gussinyer, and Diego Yeste, and Marian Albisu, and Elida Vázquez, and Arantxa Ortega, and Antonio Carrascosa
January 2009, Presse medicale (Paris, France : 1983),
Copied contents to your clipboard!