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Multiple endocrine neoplasia type 2

Disease definition

Multiple endocrine neoplasia type 2 (MEN2) is a multiple endocrine neoplasia (see this term), a polyglandular cancer syndrome characterized by the occurrence of medullary thyroid carcinoma (MTC), pheochromocytoma (PCC; see these terms), in one variant, primary hyperparathyroidism (PHPT). There are three forms: MEN2A, MEN2B, and familial medullary thyroid carcinoma (FMTC) (see these terms).

ORPHA:653

  • Synonym(s):
    • MEN2
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: D44.8
  • OMIM: 155240  162300  171400
  • UMLS: -
  • MeSH: -
  • GARD: 3830
  • MedDRA: 10028191

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