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Multiple Endocrine Neoplasia Type 1
Definition 1
Multiple endocrine neoplasia caused by inactivation of the tumor suppressor gene MEN-1. Patients may develop hyperparathyroidism and parathyroid gland adenomas, pituitary gland adenomas, pancreatic islet cell neoplasms, and carcinoid tumors. (NCI Thesaurus)
Definition 2
A rare syndrome characterized by hyperplasia and/or neoplasms of the pituitary, parathyroid glands, and pancreatic islets. Hyperparathyroidism occurs in 90% of the cases and is usually the first manifestation of the syndrome. The most frequent pancreatic manifestation is gastrinoma typically leading to Zollinger-Ellison Syndrome. The appearance of this condition has been limited to the loss of allelic heterozygosity at the 11q13 locus on the long arm of chromosome 11. Patients overall exhibit long survival times. Chemotherapy is rare and surgical management is generally dependent on the genetic expression in individual patients. (From Holland et al., Cancer Medicine, 3d ed, pp1169-72) (NLM, Medical Subject Headings)
Definition 3
A rare, inherited disorder that affects the endocrine glands and can cause tumors in the parathyroid and pituitary glands and the pancreas. These tumors are usually benign (not cancer). They cause the glands to secrete high levels of hormones, which can lead to other medical problems, such as kidney stones, fertility problems, and severe ulcers. In some cases, tumors inside the pancreas can become malignant (cancer). (NCI Dictionary)
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