Summary
Definition
History and exam
Key diagnostic factors
- diarrhea
- flushing
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 tests
1st tests to order
- serum chromogranin A/B
- urinary 5-hydroxyindoleacetic acid
- metabolic panel
- liver function tests
- complete blood count
Tests 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 metaiodobenzylguanidine (MIBG) scintigraphy
- histology
- PET scan
Treatment algorithm
localized 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.
Differentials
- Irritable bowel syndrome
- Crohn disease
- Menopause
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