Summary
Definition
History and exam
Key diagnostic factors
- diarreia
- rubor
Other diagnostic factors
- palpitações
- cólicas abdominais
- telangiectasia
- sinais de insuficiência cardíaca direita
- sopros cardíacos
- hepatomegalia
- sibilo
- pelagra
- massas abdominais
Risk factors
- síndrome da neoplasia endócrina múltipla genética tipo 1 (NEM-1)
Diagnostic tests
1st tests to order
- cromogranina A/B sérica
- ácido 5-hidroxi-indolacético urinário
- perfil metabólico
- testes da função hepática
- hemograma completo
Tests to consider
- tomografia computadorizada (TC) do tórax, abdome e pelve com duas fases hepáticas
- broncoscopia
- endoscopia
- cintilografia com receptores de somatostatina ± TC por emissão de fóton único (SPECT) de somatostatina
- cintilografia com iodo I-123 metaiodobenzilguanidina (MIBG)
- histologia
- tomografia por emissão de pósitrons com receptor de somatostatina (SSTR-PET) ou 18-fluordesoxiglucose (FDG)
Treatment algorithm
doença localizada
doença metastática
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 been part of the speakers bureau for Ipsen, Novartis, Terumo and ITM.
Martyn Caplin, BSc, DM, FRCP
Professor
Consultant in Hepatobiliary and Gastroenterology
Neuroendocrine Tumour Unit
Royal Free Hospital
London
UK
Disclosures
MC has received speaker honoraria from and is on the advisory boards for Novartis, Ipsen, Lexicon, and Pfizer.
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.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publicaton].Full text
North American Neuroendocrine Tumor Society. NANETS guidelines. 2022 [internet publication].Full text
Grozinsky-Glasberg S, Davar J, Hofland J, et al. European Neuroendocrine Tumor Society (ENETS) 2022 guidance paper for carcinoid syndrome and carcinoid heart disease. J Neuroendocrinol. 2022 Jul;34(7):e13146.Full text
Perez K, Del Rivero J, Kennedy EB, et al. Symptom management for well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO guideline. JCO Oncol Pract. 2025 May 9;:OP2500133.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials
- Síndrome do intestino irritável (SII)
- Doença de Crohn
- Menopausa
More DifferentialsGuidelines
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- Symptom management for well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO guideline
More GuidelinesPatient information
Câncer de intestino: devo fazer rastreamento?
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