PMID- 8675591
OWN - NLM
STAT- MEDLINE
DCOM- 19960815
LR  - 20181130
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 81
IP  - 7
DP  - 1996 Jul
TI  - Spectrum of pituitary disease in multiple endocrine neoplasia type 1 (MEN 1): 
      clinical, biochemical, and radiological features of pituitary disease in a large 
      MEN 1 kindred.
PG  - 2642-6
AB  - Prolactinomas and somatotropinomas are reported to be the pituitary lesions most 
      frequently associated with multiple endocrine neoplasia type 1 (MEN 1). However, 
      few reports have documented the full spectrum of pituitary disease in this 
      condition. We report herein the clinical, biochemical (PRL, alpha-subunit, 
      insulin-like growth factor-I, cortisol, and thyroid function), and radiological 
      (magnetic resonance imaging and computerized tomography scan) characteristics of 
      pituitary disease occurring in a single MEN 1 pedigree containing 165 MEN 
      1-affected members. Pituitary lesions were detected in 30 (18%) of 165 patients 
      overall. In the subgroup of MEN 1 patients (n = 131) living after recognition of 
      MEN 1 in the kindred, pituitary lesions were detected in 25 (19%). In 76% of 
      patients with pituitary lesions, the diagnosis was made by prospective screening; 
      the remainder sought medical attention for symptomatic pituitary disease. 
      Prolactinomas accounted for 76%, and nonfunctioning adenomas accounted for the 
      remaining 24%. alpha-Subunit elevation was observed in 29% of 41 patients tested, 
      and an aggressive alpha-subunit secreting macroadenoma developed in 1 subject 
      with a previously documented prolactinoma. Progression of pituitary disease 
      occurred in 47% of patients with prolactinoma. There were no cases of Cushing's 
      disease, thyrotropinoma, or somatotropinoma. We conclude that 1) in addition to 
      prolactinomas, nonfunctioning pituitary tumors are common in MEN 1; 2) 
      alpha-subunit hypersecretion is frequent in MEN 1; 3) comprehensive screening may 
      identify many clinically significant but asymptomatic pituitary lesions; and 4) 
      prolactinomas occurring in MEN 1 may behave more aggressively than sporadic 
      prolactinomas.
FAU - Burgess, J R
AU  - Burgess JR
AD  - Department of Diabetes and Endocrine Services, 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  - Journal Article
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 9002-62-4 (Prolactin)
SB  - IM
MH  - Adenoma/diagnostic imaging/genetics/physiopathology
MH  - Adult
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnostic imaging/*genetics/*physiopathology
MH  - Pituitary Gland/physiopathology
MH  - Pituitary Neoplasms/diagnostic imaging/*genetics/*physiopathology
MH  - Prolactin/metabolism
MH  - Prolactinoma/diagnostic imaging/genetics/physiopathology
MH  - Prospective Studies
MH  - Radiography
MH  - Retrospective Studies
MH  - Tasmania
EDAT- 1996/07/01 00:00
MHDA- 1996/07/01 00:01
CRDT- 1996/07/01 00:00
PHST- 1996/07/01 00:00 [pubmed]
PHST- 1996/07/01 00:01 [medline]
PHST- 1996/07/01 00:00 [entrez]
AID - 10.1210/jcem.81.7.8675591 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 1996 Jul;81(7):2642-6. doi: 10.1210/jcem.81.7.8675591.