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Enteropancreatic malignancy associated with multiple endocrine neoplasia type 1 - Risk factors and pathogenesis


Burgess, JR and Greenaway, TM and Parameswaran, V and Challis, DR and David, R and Shepherd, JJ, Enteropancreatic malignancy associated with multiple endocrine neoplasia type 1 - Risk factors and pathogenesis, Cancer, 83, (3) pp. 428-434. ISSN 0008-543X (1998) [Refereed Article]

DOI: doi:10.1002/(SICI)1097-0142(19980801)83:3<428::AID-CNCR10>3.0.CO;2-Y


BACKGROUND. Enteropancreatic malignancy is an important cause of morbidity and mortality associated with multiple endocrine neoplasia type 1 (MEN 1). How ever, 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.

Item Details

Item Type:Refereed Article
Research Division:Biomedical and Clinical Sciences
Research Group:Oncology and carcinogenesis
Research Field:Oncology and carcinogenesis not elsewhere classified
Objective Division:Health
Objective Group:Clinical health
Objective Field:Clinical health not elsewhere classified
UTAS Author:Burgess, JR (Professor John Burgess)
UTAS Author:Greenaway, TM (Dr Tim Greenaway)
UTAS Author:Parameswaran, V (Associate Professor Venkat Parameswaran)
UTAS Author:David, R (Mr Reuben David)
UTAS Author:Shepherd, JJ (Professor Joseph Shepherd)
ID Code:13238
Year Published:1998
Web of Science® Times Cited:34
Deposited By:Medicine
Deposited On:1998-08-01
Last Modified:2011-08-08

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