Multiple endocrine neoplasia type 1

Definition

An autosomal dominant tumor predisposition syndrome caused by pathogenic variants in the MEN1 gene, characterized by an increased risk of tumors of the parathyroid glands, pituitary gland, and foregut neuroendocrine tumors (most commonly pancreatic islet cells).

Also known as MEA type 1, MEA type I, men 1, men type 1, men type I, MEN1, MEN1 multiple endocrine neoplasia, MEN1 syndrome, MEN1-related multiple endocrine neoplasia, multiple endocrine adenomatosis type 1, multiple endocrine adenomatosis type I, multiple endocrine adenomatosis, type I, multiple endocrine neoplasia 1, multiple endocrine neoplasia caused by mutation in MEN1, multiple endocrine neoplasia type 1, multiple endocrine neoplasia type 1 syndrome, multiple endocrine neoplasia type I, multiple endocrine neoplasia, type I, Wermer syndrome, Wermer's syndrome — per MONDO

Also identified as

• DOID 10017 per MONDO
• ICD10CM E31.21 per MONDO
• ICD9 237.4 per MONDO
• ICD9 258.01 per MONDO
• MESH D018761 per MONDO
• NCIT C3225 per MONDO
• OMIM 131100 per MONDO
• Orphanet 652 per MONDO
• SCTID 30664006 per MONDO
• UMLS C0025267 per MONDO

Affected Organs