PMID- 8644767
OWN - NLM
STAT- MEDLINE
DCOM- 19960715
LR  - 20190627
IS  - 0002-9343 (Print)
IS  - 0002-9343 (Linking)
VI  - 100
IP  - 5
DP  - 1996 May
TI  - Somatotrophinomas in multiple endocrine neoplasia type 1: a review of clinical 
      phenotype and insulin-like growth factor-1 levels in a large multiple endocrine 
      neoplasia type 1 kindred.
PG  - 544-7
AB  - PURPOSE: Within the spectrum of pituitary disease in multiple endocrine neoplasia 
      type 1 (MEN-1), widely disparate prevalence rates for somatotrophinomas have been 
      described. Studies that combine multiple, small MEN-1 kindreds report pituitary 
      disease in 60% to 65% of patients, somatotrophinomas accounting for 27% to 37% of 
      total pituitary lesions. However, reports based on large MEN-1 family screening 
      programs have produced lower prevalence rates for pituitary adenomas (9% to 40%), 
      of which somatotrophinomas comprise up to 14%. We sought to determine the 
      prevalence of both biochemical and clinically overt growth hormone (GH) 
      hypersecretion in the largest reported MEN-1 genealogy, the Tasman 1 kindred. 
      PATIENTS AND METHODS: The Tasman 1 MEN-1 kindred contains 165 members with 
      established MEN-1. We reviewed the records of 124 MEN-1 patients for evidence of 
      acromegaly or gigantism. To determine if clinical criteria underestimate the 
      occurrence of biochemical GH hypersecretion, a subset of 33 patients was assessed 
      for elevated levels of serum insulin-like growth factor-1 (IGF-1). RESULTS: No 
      cases of acromegaly or gigantism were detected in the 124 patients reviewed. Of 
      the 33 patients screened with IGF-1, 13 had previously diagnosed pituitary 
      lesions--11 prolactinomas and 2 nonsecretory lesions. The IGF-1 levels were 
      normal in all patients studied. There were no significant differences in mean 
      IGF-1 values between patients with and without pituitary lesions. CONCLUSIONS: 
      This report represents the largest study of growth hormone secretion patterns 
      thus far described in MEN-1. The apparent absence of somatotrophinomas in a 
      kindred of this size is unexpected. These results support the existence of 
      kindred-specific MEN-1 phenotypes. We conclude that the pathogenesis of 
      GH-secreting adenomas in MEN-1 is influenced by secondary factors acting in 
      synergy with the well-documented primary MEN-1 gene defect on chromosome 11q13.
FAU - Burgess, J R
AU  - Burgess JR
AD  - Department of Diabetes, Royal Hobart Hospital, Tasmania, Australia.
FAU - Shepherd, J J
AU  - Shepherd JJ
FAU - Parameswaran, V
AU  - Parameswaran V
FAU - Hoffman, L
AU  - Hoffman L
FAU - Greenaway, T M
AU  - Greenaway TM
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PL  - United States
TA  - Am J Med
JT  - The American journal of medicine
JID - 0267200
RN  - 67763-96-6 (Insulin-Like Growth Factor I)
RN  - 9002-72-6 (Growth Hormone)
SB  - IM
MH  - Adenoma/epidemiology/*genetics/metabolism
MH  - Adult
MH  - Female
MH  - Growth Hormone/*metabolism
MH  - Humans
MH  - Insulin-Like Growth Factor I/*analysis
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*genetics
MH  - Phenotype
MH  - Pituitary Neoplasms/epidemiology/*genetics/metabolism
MH  - Prevalence
MH  - Prolactinoma/epidemiology/genetics
MH  - Tasmania/epidemiology
EDAT- 1996/05/01 00:00
MHDA- 1996/05/01 00:01
CRDT- 1996/05/01 00:00
PHST- 1996/05/01 00:00 [pubmed]
PHST- 1996/05/01 00:01 [medline]
PHST- 1996/05/01 00:00 [entrez]
AID - S0002934396000125 [pii]
AID - 10.1016/s0002-9343(96)00012-5 [doi]
PST - ppublish
SO  - Am J Med. 1996 May;100(5):544-7. doi: 10.1016/s0002-9343(96)00012-5.