Multiple endocrine neoplasia type 1

J Surg Oncol. 2005 Mar 1;89(3):143-50. doi: 10.1002/jso.20181.

Abstract

Multiple endocrine neoplasia-1 (MEN-1) is an autosomal dominant inherited syndrome that occurs due to inactivating mutations of the MEN1 gene locus, coding for a tumor-suppressor protein, menin. The components of MEN-1 are hyperparathyroidism due to multiple parathyroid adenomas, pancreatic neuroendocrine tumors, and pituitary adenomas, in addition to some less common neoplastic manifestations. Care of people with MEN-1 requires knowledge of the problems that may arise, and the best approaches to detect and care for the manifestations of this incurable, but manageable, disease.

Publication types

  • Review

MeSH terms

  • Adenoma / diagnosis
  • Endosonography
  • Female
  • Genes, Tumor Suppressor*
  • Humans
  • Hyperparathyroidism / etiology
  • Insulinoma / diagnostic imaging
  • Insulinoma / surgery
  • Male
  • Multiple Endocrine Neoplasia Type 1* / diagnosis
  • Multiple Endocrine Neoplasia Type 1* / genetics
  • Multiple Endocrine Neoplasia Type 1* / surgery
  • Neuroectodermal Tumors / diagnostic imaging
  • Pancreatic Neoplasms / diagnostic imaging
  • Pancreatic Neoplasms / surgery
  • Parathyroid Neoplasms / diagnosis
  • Pituitary Neoplasms / surgery
  • Prolactinoma / surgery
  • Proto-Oncogene Proteins / genetics*
  • Tomography, Emission-Computed, Single-Photon
  • Tomography, X-Ray Computed
  • Vipoma / surgery

Substances

  • MEN1 protein, human
  • Proto-Oncogene Proteins