Last reviewed:December 2019
Last updated:January  2018

Summary

Definition

History and exam

Key diagnostic factors

  • young age (MEN1/2)
  • positive family history (MEN1/2)
  • episodic triad of sweating, palpitations, and headache (MEN2)
  • clinical features of kidney stones (MEN1/2)
  • facial angiofibromas or collagenomas (MEN1)
  • mucosal neuromas (MEN2B)
  • arm span and upper-to-lower-body-segment ratio (MEN2B)
  • palpable thyroid nodule (MEN2)
  • irregular menses (MEN1)
  • visual changes (MEN1)
  • unexplained flushing (MEN2)
  • infertility (MEN1)
  • clinical features of acromegaly (MEN1)
  • clinical features of thyrotoxicosis (MEN1)

Other diagnostic factors

  • weight changes (MEN1/2)
  • hypertension (MEN1/2)
  • abdominal pain (MEN1/2)
  • headache (MEN1/2)
  • low-trauma fractures (MEN1)
  • altered bowel habit (MEN1/2)
  • palpitations (MEN1/2)
  • easy bruising (MEN1/2)
  • slow wound healing (MEN1/2)
  • erectile dysfunction (MEN1)
  • clinical features of hypercortisolism/Cushing syndrome (MEN1/2)
  • anxiety (MEN1/2)
  • heat intolerance (MEN1)
  • confusion (MEN1/2)
  • dehydration (MEN1/2)
  • gastrointestinal bleeding (MEN2)
  • hepatomegaly (MEN2)

Risk factors

  • familial cases of MEN
  • RET proto-oncogene mutation
  • MEN1 (menin) mutation

Diagnostic investigations

Treatment algorithm

Contributors

Research Registrar

Department of Endocrinology

Barts and the London NHS Trust and Queen Mary University of London

London

UK

Disclosures

RG declares that she has no competing interests.

Senior Lecturer & Honorary Consultant

Department of Endocrinology

Barts and the London Medical School

London

UK

Disclosures

MD has been reimbursed for travel expenses and conference attendance for ENETS (European Neuroendocrine Tumour Society) by Novartis.

Dr Rebecca Gorrigan and Dr Maralyn Druce would like to gratefully acknowledge Dr Jennifer Mammen and Dr Roberto Salvatori, previous contributors to this monograph. JM and RS declare that they have no competing interests.

Peer reviewersVIEW ALL

Director

Division of Endocrinology and Diabetes

The Children's Hospital of Philadelphia

Philadelphia

PA

Disclosures

ML declares that he has no competing interests.

Associate Professor of Endocrinology

Catholic University School of Medicine

Rome

Italy

Disclosures

SC declares that he has no competing interests.

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