Multiple endocrine neoplasia: an update

Intern Med J. 2019 Aug;49(8):954-961. doi: 10.1111/imj.14394.

Abstract

The multiple endocrine neoplasia (MEN) syndromes include MEN1, MEN2 (formerly MEN2A), MEN3 (formerly MEN2B) and the recently identified MEN4. Clinical presentations are varied and often relate to the overproduction of specific hormones. Understanding the genetics of each syndrome assists in determining screening timelines. Treatments for each manifestation are dependent on location, risk of recurrence or malignancy, hormone excess and surgical morbidity. Multidisciplinary management should include geneticists, genetic counsellors, endocrinologists and endocrine surgeons.

Keywords: genetics; multiple endocrine neoplasia; screening.

Publication types

  • Review

MeSH terms

  • Combined Modality Therapy
  • Endocrine Surgical Procedures
  • Genetic Markers
  • Genetic Testing
  • Humans
  • Multiple Endocrine Neoplasia / classification
  • Multiple Endocrine Neoplasia / diagnosis*
  • Multiple Endocrine Neoplasia / genetics*
  • Multiple Endocrine Neoplasia / therapy*
  • Recurrence

Substances

  • Genetic Markers