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Multiple endocrine neoplasia type II with Zollinger-Ellison syndrome caused by a solitary pancreatic gastrinoma.

THERE are three familial forms of multiple endocrine neoplasia (MEN), all of which are inherited as autosomal dominant traits. Multiple endocrine neoplasia type I is characterized by hyperparathyroidism, pituitary tumors, pancreatic islet cell tumors, and other less common manifestations. 1,2 Multiple endocrine neoplasia type II is characterized by medullary carcinoma of the thyroid, pheochromocytoma, and hyperparathyroidism. 3,4 Patients with MEN type III, otherwise named "MEN type lib," are similar to patients with MEN type II but also have mucosal neuromas and marfanoid habitus. 4,5 These three conditions are inherited separately and are distinct entities. Thus, mucosal neuromas are restricted to MEN type IIb, pheochromocytoma and medullary thyroid carcinoma occur only in MEN type II, and pituitary tumors and islet cell tumors occur only in MEN type I. However, because increasing numbers of patients with MEN syndromes are now being followed up, together with the increasing longevity of these patients, features