Skip to main content
SpringerLink
Log in

Gastric Neuroendocrine Tumors (Carcinoids)

  • Stomach and Duodenum (J Pisegna and J Benhammou, Section Editors)
  • Published:
Current Gastroenterology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

The diagnosis of gastric neuroendocrine tumors (NETs) is being made with increased frequency likely as a result of more upper endoscopies being done for unrelated reasons. It is therefore vital that gastroenterologists become familiar with the basic work-up and management of patients found to have these tumors. This review describes the classification, pathophysiology, clinical characteristics, and treatment options of the different gastric NETs.

Recent Findings

In addition to the three traditional subtypes of gastric NETs, additional cases associated with achlorhydria and appropriate hypergastrinemia may exist. The management of gastric NETs between 1 and 2 cm in size remains controversial and needs to be individualized.

Summary

Gastric NETs are uncommon but are now diagnosed more frequently. This review highlights the role of hypergastrinemia in their development and the controversies around their management.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Díez M, Teulé A, Salazar R. Gastroenteropancreatic neuroendocrine tumors: diagnosis and treatment. Ann Gastroenterol. 2013;26(1):29–36.

    PubMed  PubMed Central  Google Scholar 

  2. Scherübl H, Cadiot G, Jensen RT, Rösch T, Stölzel U, Klöppel G. Neuroendocrine tumors of the stomach (gastric carcinoids) are on the rise. Small tumors, small problems? Endoscopy. 2010;42(8):664–71. https://doi.org/10.1055/s-0030-1255564.

    Article  PubMed  Google Scholar 

  3. 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;3(10):1335–42. https://doi.org/10.1001/jamaoncol.2017.0589.

    Article  Google Scholar 

  4. Nikou GC, Angelopoulos TP. Current concepts on gastric carcinoid tumors. Gastroenterol Res Pract. 2012;2012:287825. https://doi.org/10.1155/2012/287825.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Al-Harbi O, Shakir M, Al-Brahim N. Gastric carcinoid and obesity: association or coincidence? Report of two cases and literature review. Case Rep Gastrointest Med. 2013;2013:848075–4. https://doi.org/10.1155/2013/848075.

    Article  Google Scholar 

  6. •• Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010;39(6):707–12. https://doi.org/10.1097/MPA.0b013e3181ec124e A landmark review of the pathologic classification of neuroendocrine tumors, highlighting issues regarding stages and grades of NETs and standardizing the way NETs are classified in pathology reports.

    Article  PubMed  Google Scholar 

  7. Grozinsky-Glasberg S, Alexandraki KI, Angelousie A, Chatzellis E, Sougioultzis S, Kaltsas G. Endocrinol Metab Clin North Am. 2018;47(3):645–660. https://doi.org/10.1016/j.ecl.2018.04.013.

    Article  Google Scholar 

  8. Abraham SC, Aidan Carney J, Ooi A, Choti MA, Argani P. Achlorhydria, parietal cell hyperplasia, and multiple gastric carcinoids: a new disorder. Am J Surg Pathol. 2005;29:969–75.

    Article  Google Scholar 

  9. Waldum HL, Hauso Ø, Brenna E, Qvigstad G, Fossmark R. Does long-term profound inhibition of gastric acid secretion increase the risk of ECL cell-derived tumors in man? Scand J Gastroenterol. 2016;51(7):767–73. https://doi.org/10.3109/00365521.2016.1143527.

    Article  CAS  PubMed  Google Scholar 

  10. Nilsson O, Wängberg B, Johansson L, Modlin IM, Ahlman H. Praomys (Mastomys) natalensis: a model for gastric carcinoid formation. Yale J Biol Med. 1992;65(6):741–51.

    CAS  PubMed  PubMed Central  Google Scholar 

  11. •• Corey B, Chen H. Neuroendocrine tumors of the stomach. Surg Clin N Am. 2017;97(2):333–43. https://doi.org/10.1016/j.suc.2016.11.008 A recent review of gastric NETs that provides an algorithm for their management dependent on subtype.

    Article  PubMed  Google Scholar 

  12. Qvigstad G, Falkmer S, Westre B, Waldum HL. Clinical and histopathological tumour progression in ECL cell carcinoids (“ECLomas”). APMIS. 1999;107(12):1085–92.

    Article  CAS  Google Scholar 

  13. •• Schubert ML, Peura DA. Control of gastric acid secretion in health and disease. Gastroenterology. 2008;134(7):1842–60. https://doi.org/10.1053/j.gastro.2008.05.021 A review of the physiology and pathophysiology of acid secretion, highlighting diseases of acid hypersecretion and the mechanisms of acid-blocking medications used to treat them.

    Article  CAS  PubMed  Google Scholar 

  14. Wardlaw R, Smith JW. Gastric carcinoid tumors. Ochsner J. 2008;8(4):191–6.

    PubMed  PubMed Central  Google Scholar 

  15. Ooi A, Ota M, Katsuda S, Nakanishi I, Sugawara H, Takahashi I. An unusual case of multiple gastric carcinoids associated with diffuse endocrine cell hyperplasia and parietal cell hypertrophy. Endocr Pathol. 1995;6(3):229–37.

    Article  Google Scholar 

  16. Gibril F, Schumann M, Pace A, Jensen RT. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore). 2004;83(1):43–83.

    Article  Google Scholar 

  17. Bordi C, D’Adda T, Azzoni C, Canavese G, Brandi ML. Gastrointestinal endocrine tumors: Recent developments. Endocr Pathol. 1998;9:99–115. https://doi.org/10.1007/BF02782603.

    Article  Google Scholar 

  18. • Metz DC. Diagnosis of the Zollinger–Ellison syndrome. Clin Gastroenterol Hepatol. 2012;10(2):126–30. https://doi.org/10.1016/j.cgh.2011.07.012 A practical approach to the diagnosis, work-up, and management of ZES, with particular reference to the important clinical differences between appropriate and inappropriate hypergastrinemia.

    Article  PubMed  Google Scholar 

  19. •• Kunz PL, Reidy-Lagunes D, Anthony LB, Bertino EM, Brendtro K, Chan JA, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013;42(4):557–77. https://doi.org/10.1097/MPA.0b013e31828e34a4 In 2010, the North American Neuroendocrine Tumor Society (NANETS) published a comprehensive and updated guideline addressing the management of NETs. This article addresses controversial topics in the management of NETs.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Dias AR, Azevedo BC, Alban LBV, Yagi OK, Ramos MFKP, Jacob CE, et al. Gastric neuroendocrine tumor: review and update. Arq Bras Cir Dig. 2017;30(2):150–4. https://doi.org/10.1590/0102-6720201700020016.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Jenny HE, Ogando PA, Fujitani K, Warner RR, Divino CM. Laparoscopic antrectomy: a safe and definitive treatment in managing type 1 gastric carcinoids. Am J Surg. 2016;211(4):778–82. https://doi.org/10.1016/j.amjsurg.2015.08.040.

    Article  PubMed  Google Scholar 

  22. Lau SC, Abdel-Rahman O, Cheung WY. Improved survival with higher doses of octreotide long-acting release in gastroenteropancreatic neuroendocrine tumors. Med Oncol. 2018;35(9):123. https://doi.org/10.1007/s12032-018-1189-1.

    Article  CAS  PubMed  Google Scholar 

  23. Massironi S, Zilli A, Fanetti I, Ciafardini C, Conte D, Peracchi M. Intermittent treatment of recurrent type 1 gastric carcinoids with somatostatin analogues in patients with chronic autoimmune atrophic gastritis. Dig Liver Dis. 2015;47(11):978–83. https://doi.org/10.1016/j.dld.2015.07.155.

    Article  CAS  PubMed  Google Scholar 

  24. Fykse V, Sandvik AK, Qvigstad G, Falkmer SE, Syversen U, Waldum HL. Treatment of ECL cell carcinoids with octreotide LAR. Scand J Gastroenterol. 2004;39(7):621–8.

    Article  CAS  Google Scholar 

  25. Tomassetti P, Migliori M, Caletti GC, Fusaroli P, Corinaldesi R, Gullo L. Treatment of type II gastric carcinoid tumors with somatostatin analogues. N Engl J Med. 2000;343(8):551–4.

    Article  CAS  Google Scholar 

  26. Fossmark R, Sørdal Ø, Jianu CS, Qvigstad G, Nordrum IS, Boyce M, et al. Treatment of gastric carcinoids type 1 with the gastrin receptor antagonist netazepide (YF476) results in regression of tumours and normalisation of serum chromogranin a. Aliment Pharmacol Ther. 2012;36(11–12):1067–75. https://doi.org/10.1111/apt.12090.

    Article  CAS  Google Scholar 

  27. • Boyce M, Moore AR, Sagatun L, Parsons BN, Varro A, Campbell F, et al. Netazepide, a gastrin/cholecystokinin-2 receptor antagonist, can eradicate gastric neuroendocrine tumours in patients with autoimmune chronic atrophic gastritis. Br J Clin Pharmacol. 2017;83(3):466–75. https://doi.org/10.1111/bcp.13146 A study of netazepide, an investigational agent that shows promise in treating type I gastric NETs especially in patients who want to avoid regular endoscopies or surgery.

    Article  CAS  PubMed  Google Scholar 

  28. Delle Fave G, O’Toole D, Sundin A, Taal B, Ferolla P, Ramage JK, et al. (Vienna Consensus Conference participants). ENETS consensus guidelines update for gastroduodenal neuroendocrine neoplasms. Neuroendocrinology. 2016;103(2):119–24. https://doi.org/10.1159/000443168.

    Article  CAS  PubMed  Google Scholar 

  29. • Sato Y. Clinical features and management of type I gastric carcinoids. Clin J Gastroenterol. 2014;7:381. https://doi.org/10.1007/s12328-014-0528-9 A recent review of the pathophysiology, diagnosis, management, and prognosis of type I gastric NETs.

    Article  PubMed  Google Scholar 

  30. Sato Y, Hashimoto S, Mizuno K, Takeuchi M, Terai S. Management of gastric and duodenal neuroendocrine tumors. World J Gastroenterol. 2016;22(30):6817–28.

    Article  CAS  Google Scholar 

  31. Ravizza D, Fiori G, Trovato C, Fazio N, Bonomo G, Luca F, et al. Long-term endoscopic and clinical follow-up of untreated type 1 gastric neuroendocrine tumours. Dig Liver Dis. 2007;39(6):537–43.

    Article  CAS  Google Scholar 

  32. Scherübl H, Jensen RT, Cadiot G, Stölzel U, Klöppel G. Management of early gastrointestinal neuroendocrine neoplasms. World J Gastrointest Endosc. 2011;3(7):133–9. https://doi.org/10.4253/wjge.v3.i7.133.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Ito T, Igarashi H, Uehara H, Berna MJ, Jensen RT. Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors. Medicine (Baltimore). 2013;92(3):135–81.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Hospital of the University of Pennsylvania, Perelman School of Medicine, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Craig R. Gluckman & David C. Metz

Authors
  1. Craig R. Gluckman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. David C. Metz
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to David C. Metz.

Ethics declarations

Conflict of Interest

David Metz reports personal fees from Ipsen, Novartis, Lexicon, and Takeda and grants from AAA and Lexicon and Emeritus chair of NANETS. Craig Gluckman has nothing to disclose.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Stomach and Duodenum

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Gluckman, C.R., Metz, D.C. Gastric Neuroendocrine Tumors (Carcinoids). Curr Gastroenterol Rep 21, 13 (2019). https://doi.org/10.1007/s11894-019-0684-7

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11894-019-0684-7

Keywords

Search

Navigation