Gastrinoma

Stuart D. Wilson, MD

A gastrinoma is an endocrine tumor that elaborates the hormone gastrin. The consequences of the hypergastrinemia are gastric acid hypersecretion and related complications. Before the identification of the hormone gastrin as the gastric secretagogue, the tumors were called "ulcerogenic tumors of the pancreas."1,2 Zollinger-Ellison syndrome, or Z-E syndrome, is used frequently in the medical literature in place of the term gastrinoma.3

Gastrinoma patients should be classified as having either sporadic or familial gastrinoma. This distinction is important because the pathophysiology, natural history, and medical or surgical management of sporadic and familial gastrinoma patients are different. Sporadic gastrinomas are not inherited and are only rarely associated with other endocrinopathies. Familial gastrinomas are those that develop in individuals who have inherited the genetic trait for multiple endocrine neoplasia type 1 (MEN l).4 The eponym for the MEN 1 syndrome is Wermer's syndrome.5 The term multiple endocrine adenopathy type 1 (MEA-1) is preferred to MEN 1 by some authors.4'6 In this chapter sporadic gastrinomas are discussed. Familial gastrinomas, occurring in patients with MEN 1, are discussed in Chapter 76.

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