Carcinoid syndrome

Last reviewed: 29 Oct 2022
Last updated: 26 Jul 2022

Summary

Definition

History and exam

Key diagnostic factors

  • diarrhoea
  • flushing
More key diagnostic factors

Other diagnostic factors

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

Risk factors

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

Diagnostic investigations

1st investigations to order

  • serum chromogranin A/B
  • urinary 5-hydroxyindoleacetic acid
  • metabolic panel
  • liver function tests
  • full blood count
More 1st investigations to order

Investigations to consider

  • CT chest, abdomen, and pelvis with dual-phase liver
  • bronchoscopy
  • endoscopy
  • somatostatin receptor scintigraphy ± somatostatin single photon emission CT (SPECT)
  • iodine I-123 meta-iodobenzylguanidine (MIBG) scintigraphy
  • histology
  • PET scan
More investigations to consider

Treatment algorithm

ONGOING

localised disease

metastatic disease

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 AAA, Novartis and Ipsen, and has given consultancy advice to Pfizer and Ipsen. RS has engaged in the speaker's bureau for AAA, Ipsen, and Mylan.

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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