Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Sep;28(5):336-348.
doi: 10.1159/000512089. Epub 2021 Jan 20.

Neuroendocrine Tumors of the Gastrointestinal Tract: A Focused Review and Practical Approach for Gastroenterologists

Affiliations
Review

Neuroendocrine Tumors of the Gastrointestinal Tract: A Focused Review and Practical Approach for Gastroenterologists

Joana Carvão et al. GE Port J Gastroenterol. 2021 Sep.

Abstract

Neuroendocrine tumors (NETs) are rare tumors derived from the neuroendocrine cell system, and more commonly found in the gastrointestinal (GI) tract. Over the last decades, the incidence of GI-NETs has been steadily increasing, partly due to the expanding indications for endoscopy. Most patients with NETs are asymptomatic, and their NETs are noticed during screening examinations; thus, endoscopists are on the frontline of the diagnosis of GI-NETs. Since GI-NETs are less frequent than other malignancies, the natural history, diagnosis, and management of these tumors may not be fully understood. In this review, we aim to update the endoscopist on key clinical features and management of patients with gastric, duodenal, and rectal NETs.

Os tumores neuroendócrinos (TNE) são tumores raros derivados do sistema neuroendócrino e mais frequentemente encontrados no trato gastrointestinal. Nas últimas décadas, a incidência de TNEs gastrointestinais tem vindo a aumentar de forma consistente, em parte devido às crescentes indicações da endoscopia. De facto, a maioria dos doentes com TNEs são assintomáticos e a lesão é identificada durante procedimentos de rastreio. Os endoscopistas estão na linha de frente do diagnóstico destes tumores. Como os TNEs são menos frequentemente encontrados em comparação com outras neoplasias, a história natural, o diagnóstico e a abordagem terapèutica destes tumores podem não ser totalmente compreendidos. Nesta revisão, os autores têm como objetivo atualizar o endoscopista sobre as principais características clínicas e abordagem endoscópica de doentes com TNE gástricos, duodenais e rectais.

Keywords: Endoscopy; Gastrointestinal tract; Neuroendocrine tumors.

PubMed Disclaimer

Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
Multiple, small (<1–2 cm), reddish, polypoid lesion in the stomach corpus with surrounding atrophic mucosa suggestive of type I g-NET.
Fig. 2
Fig. 2
Single and large (>1 cm) lesion in the stomach corpus with depressed center, consistent with type III g-NET.
Fig. 3
Fig. 3
Summarized management of gastric NETs, according to authors' own experience/preference. * The management of type II is similar to type I with the exception that we favor endoscopic resection of all visible tumors; consider ER of NETs with atypical/irregular features; # or ESD if depressed/atypical features); NET, neuroendocrine tumor; EMR, endoscopic mucosal resection; ESD, endoscopic submucosal dissection; FTR, full-thickness resection.
Fig. 4
Fig. 4
Summarized management of non-functioning duodenal NETs, according to authors' own experience/preference. If periampullary location, surgical resection is recommended regardless of size; endoscopic resection may be performed in very selected cases. NET, neuroendocrine tumor; EMR, endoscopic mucosal resection; ESD, endoscopic submucosal dissection.
Fig. 5
Fig. 5
Summarized management of rectal NETs, according to authors' own experience/preference. * ESD, EFTR, or TEMS alternative if no muscular invasion and patient refuses major surgery; # anterior resection + total mesorectal excision or abdominoperineal resection; surgery/stent/chemotherapy. EMR, endoscopic mucosal resection; ESD, endoscopic submucosal dissection; EFTR, endoscopic full-thickness resection; TEMS, transanal endoscopic microsurgery.

Similar articles

Cited by

References

    1. Wang R, Zheng-Pywell R, Chen HA, Bibb JA, Chen H, Rose JB. Management of Gastrointestinal Neuroendocrine Tumors. Clin Med Insights Endocrinol Diabetes. 2019 Oct;12:1179551419884058. - PMC - PubMed
    1. Niederle MB, Hackl M, Kaserer K, Niederle B. Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters. Endocr Relat Cancer. 2010 Oct;17((4)):909–18. - PubMed
    1. Dasari A, Shen C, Halperin D, Zhao B, Zhou S, Xu Y, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017 Oct;3((10)):1335–42. - PMC - PubMed
    1. Nagtegaal ID, Odze RD, Klimstra D, Paradis V, Rugge M, Schirmacher P, WHO Classification of Tumours Editorial Board et al. The 2019 WHO classification of tumours of the digestive system. Histopathology. 2020 Jan;76((2)):182–8. - PMC - PubMed
    1. Öberg K, Knigge U, Kwekkeboom D, Perren A, ESMO Guidelines Working Group Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012 Oct;23(Suppl 7):vii124–30. - PubMed