E31.2

Multiple endocrine neoplasia [MEN] syndromes

A group of autosomal dominant diseases characterized by the combined occurrence of tumors involving two or more endocrine glands that secrete peptide hormones or amines. These neoplasias are often benign but can be malignant. They are classified by the endocrine glands involved and the degree of aggressiveness. The two major forms are men1 and men2 with gene mutations on chromosome 11 and chromosome 10, respectively.

An autosomal dominant inherited neoplastic syndrome characterized by the development of various endocrine neoplasms and abnormalities in various anatomic sites. There are three types recognized: type 1 men 1), caused by inactivation of the tumor suppressor gene men-1, type 2a men 2a), caused by mutation of the ret gene, and type 2b men 2b) also caused by mutation of the ret gene. Patients with men 1 may develop hyperparathyroidism and parathyroid gland adenomas, pituitary gland adenomas, pancreatic islet cell neoplasms, and carcinoid tumors. Patients with men 2a develop medullary thyroid carcinomas, and may also develop pheochromocytomas and parathyroid gland hyperplasia. Patients with men 2b develop medullary thyroid carcinomas and numerous neural defects including neuromas.

An inherited condition that may result in the development of cancers of the endocrine system. There are several types of multiple endocrine neoplasia syndrome, and patients with each type may develop different types of cancer. The altered genes that cause each type can be detected with a blood test.

Group of specific, familial syndromes characterized by simultaneous neoplastic transformation of multiple endocrine tissues, typically the parathyroid glands, pancreatic islets, and anterior pituitary.

E31.2 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.

This is the American ICD-10-CM version of E31.2 – other international versions of ICD-10 E31.2 may differ.