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

✍ Scribed by John R. Burgess; Tim M. Greenaway; Venkateswaran Parameswaran; David R. Challis; Rueben David; Joseph J. Shepherd

• Publisher: John Wiley and Sons
• Year: 1998
• Tongue: English
• Weight: 86 KB
• Volume: 83
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/(sici)1097-0142(19980801)83:3<428::aid-cncr10>3.0.co;2-y

✦ Synopsis

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.