RT Journal
A1 Maton PN, Norton JA, Nieman LK, Doppman JL, Jensen RT
T1 MUltiple endocrine neoplasia type ii with zollinger-ellison syndrome caused by a solitary pancreatic gastrinoma
JF JAMA
JO JAMA
YR 1989
FD July 28
VO 262
IS 4
SP 535
OP 537
DO 10.1001/jama.1989.03430040107035
UL http://dx.doi.org/10.1001/jama.1989.03430040107035
AB 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