PMID- 9690534
OWN - NLM
STAT- MEDLINE
DCOM- 19980813
LR  - 20190620
IS  - 0008-543X (Print)
IS  - 0008-543X (Linking)
VI  - 83
IP  - 3
DP  - 1998 Aug 1
TI  - Enteropancreatic malignancy associated with multiple endocrine neoplasia type 1: 
      risk factors and pathogenesis.
PG  - 428-34
AB  - BACKGROUND: Enteropancreatic malignancy is an important cause of morbidity and 
      mortality associated with multiple endocrine neoplasia type 1 (MEN 1). However, 
      the risk factors and mechanisms of the tumorigenesis of this malignancy are 
      poorly understood. METHODS: The authors conducted a retrospective study of 
      factors associated with the development of malignant enteropancreatic tumor in 69 
      patients with MEN 1 belonging to a single family. RESULTS: Metastatic 
      enteropancreatic tumor and gastrinoma were identified in 20% and 36% of patients, 
      respectively. Compared with MEN 1 patients who did not have an immediate family 
      history of enteropancreatic malignancy, MEN 1 patients with a first-degree 
      relative affected by enteropancreatic malignancy had an increased risk of 
      developing disseminated tumor (odds ratio, 3.7; P < 0.05). In addition, 
      hypergastrinemia and advanced age were both associated with a significant 
      increase in the risk of enteropancreatic malignancy. Elevated serum glycoprotein 
      alpha subunit levels were associated with enterochromaffin-like cell hyperplasia, 
      gastric carcinoid formation, and disseminated enteropancreatic tumor in 
      hypergastrinemic patients (P < 0.05). CONCLUSIONS: Disease modifier factors act 
      in concert with the MEN 1 gene to modulate the development of enteropancreatic 
      neoplasia. It is possible to identify MEN 1 patients at high risk for developing 
      aggressive enteropancreatic tumors. Heritable disease modifier factor(s) 
      affecting enteropancreatic malignancy appear to reside at loci distinct from that 
      of the MEN 1 gene.
FAU - Burgess, J R
AU  - Burgess JR
AD  - Royal Hobart Hospital, Australia. jburges@postoffice.utas.edu.au
FAU - Greenaway, T M
AU  - Greenaway TM
FAU - Parameswaran, V
AU  - Parameswaran V
FAU - Challis, D R
AU  - Challis DR
FAU - David, R
AU  - David R
FAU - Shepherd, J J
AU  - Shepherd JJ
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Cancer
JT  - Cancer
JID - 0374236
RN  - 0 (Gastrins)
SB  - IM
MH  - Adenoma/etiology
MH  - Adult
MH  - Female
MH  - Gastrinoma/*etiology
MH  - Gastrins/blood
MH  - Humans
MH  - Hyperparathyroidism/etiology/therapy
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*complications
MH  - Neoplasm Metastasis
MH  - Pancreatic Neoplasms/*etiology
MH  - Retrospective Studies
MH  - Risk Factors
EDAT- 1998/08/05 02:04
MHDA- 2000/06/20 09:00
CRDT- 1998/08/05 02:04
PHST- 1998/08/05 02:04 [pubmed]
PHST- 2000/06/20 09:00 [medline]
PHST- 1998/08/05 02:04 [entrez]
AID - 10.1002/(SICI)1097-0142(19980801)83:3<428::AID-CNCR10>3.0.CO;2-Y [pii]
AID - 10.1002/(sici)1097-0142(19980801)83:3<428::aid-cncr10>3.0.co;2-y [doi]
PST - ppublish
SO  - Cancer. 1998 Aug 1;83(3):428-34. doi: 
      10.1002/(sici)1097-0142(19980801)83:3<428::aid-cncr10>3.0.co;2-y.