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Enteropancreatic malignancy associated with multiple endocrine neoplasia type 1 - Risk factors and pathogenesis
journal contribution
posted on 2023-05-16, 11:04 authored by John BurgessJohn Burgess, Timothy GreenawayTimothy Greenaway, Venkatesh ParameswaranVenkatesh Parameswaran, Challis, DR, David, R, Shepherd, JJBACKGROUND. 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.
History
Publication title
CancerVolume
83Pagination
428-434ISSN
0008-543XDepartment/School
Tasmanian School of MedicinePublisher
John Wiley & Sons IncPlace of publication
USARepository Status
- Restricted