PMID- 15761658 OWN - NLM STAT- MEDLINE DCOM- 20050705 LR - 20181201 IS - 1386-341X (Print) IS - 1386-341X (Linking) VI - 7 IP - 2 DP - 2004 TI - Isolated familial somatotropinoma. PG - 95-101 AB - The great majority of growth hormone (GH)-secreting pituitary tumors are sporadic, though a few occur with a familial aggregation, either as a component of multiple endocrine neoplasia, type 1 (MEN1) or Carney Complex, or when unassociated with other tumors, as isolated familial somatotropinomas (IFS). This report reviews the clinical and genetic information available from the 46 families reported to date with the latter syndrome. In contrast to sporadic tumors, GH-secreting tumors in IFS occur at an earlier age, especially when all affected family members are from a single generation. The IFS gene is believed to be a tumor suppressor gene, based on the presence of loss of heterozygosity. Although the gene still remains to be identified there is strong evidence for linkage to a locus of less than 10 Mb on chromosome 11q13.1-13.3. FAU - Soares, Beatriz Santana AU - Soares BS AD - Section of Endocrinology and Metabolism, University of Illinois at Chicago, IL 60612, USA. FAU - Frohman, Lawrence A AU - Frohman LA LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Pituitary JT - Pituitary JID - 9814578 RN - 12629-01-5 (Human Growth Hormone) SB - IM MH - Acromegaly/etiology MH - Adolescent MH - Adult MH - Child MH - Chromosome Mapping MH - *Chromosomes, Human, Pair 11 MH - Chromosomes, Human, Pair 17 MH - Chromosomes, Human, Pair 2 MH - Family MH - Female MH - Haplotypes MH - Human Growth Hormone/*metabolism MH - Humans MH - Male MH - Middle Aged MH - Pituitary Neoplasms/*genetics/metabolism MH - Recombination, Genetic EDAT- 2005/03/12 09:00 MHDA- 2005/07/06 09:00 CRDT- 2005/03/12 09:00 PHST- 2005/03/12 09:00 [pubmed] PHST- 2005/07/06 09:00 [medline] PHST- 2005/03/12 09:00 [entrez] AID - 10.1007/s11102-005-0-04-0 [doi] PST - ppublish SO - Pituitary. 2004;7(2):95-101. doi: 10.1007/s11102-005-0-04-0.