Last reviewed: 21 Aug 2021
Last updated: 22 Feb 2018

Summary

Definition

History and exam

Other diagnostic factors

  • palpitations
  • abdominal cramps
  • telangiectasia
  • signs of right heart failure
  • cardiac murmurs
  • hepatomegaly
  • wheeze
  • pellagra
  • abdominal masses

Risk factors

  • genetic multiple endocrine neoplasia type 1 (MEN-1) syndrome

Diagnostic investigations

1st investigations to order

  • serum chromogranin A/B
  • urinary 5-hydroxyindoleacetic acid
  • metabolic panel
  • liver function tests
  • complete blood count

Investigations to consider

  • computed tomography (CT) chest, abdomen, and pelvis with dual-phase liver
  • bronchoscopy
  • endoscopy
  • somatostatin receptor scintigraphy ± somatostatin single photon emission computer tomography (SPECT)
  • iodine I-123 metaiodobenzylguanidine (MIBG) scintigraphy
  • histology
  • positive emission tomography (PET) scan

Treatment algorithm

Contributors

Authors

Rajaventhan Srirajaskanthan, BSc (Hons), MD (Res), FRCP

Consultant Gastroenterologist

Neuroendocrine Tumour Unit

Kings College Hospital

London

UK

Disclosures

RS has received research grants from Novartis and Ipsen, and has has given consultancy advice to Pfizer and Ipsen.

Martyn Caplin, BSc, DM, FRCP

Professor

Consultant in Hepatobiliary and Gastroenterology

Neuroendocrine Tumour Unit

Royal Free Hospital

London

UK

Disclosures

MC is on the advisory boards for Novartis, Ipsen, Lexicon, and Pfizer; he has also received speaker honoraria from them.

Peer reviewers

Wail Malaty, MD

Clinical Professor

Department of Family Medicine

University of North Carolina

Chapel Hill

Assistant Program Director

MAHEC Rural Family Medicine Residency

Hendersonville

NC

Disclosures

WM declares that he has no competing interests.

Rajesh K. Garg, MD

Instructor in Medicine

Brigham and Women's Hospital

Division of Endocrinology

Diabetes and Hypertension

Boston

MA

Disclosures

RKG declares that he has no competing interests.

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