Etiology of Sporadic Gastrinoma Syndrome

The etiology of the sporadic gastrinoma syndrome has not been defined, nor has a specific gene defect been identified. Studies by J. Thompson's group were unable to document mutations of the p53 and ras genes in gastrinomas, but they did find amplification of HER-2/neĀ«, a protooncogene related to the epidermal growth factor receptor.46 A gene defect has been localized to the long arm of chromosome 11 in patients with MEN 1, some of whom acquire gastrinomas, but patients with sporadic gastrinomas do not appear to have this defect.47

Chronic alcohol abuse may be an important risk factor in the genesis of some gastrinomas of the sporadic type. A history of excessive alcohol intake (>50 g/day) that antedated the Z-E syndrome in 23 of 36 (64%) sporadic gastrinoma patients admitted to our clinical research center could be documented over a 30-year period. Gastrinomas in the duodenal wall and in lymph nodes were a common finding in this group of patients, whereas primary pancreatic gastrinomas were rare. Death from progressive tumor growth has been infrequent. These observations suggest that the Z-E syndrome is not a single disease process but that several subgroups exist.48

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