Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Gastrinoma

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_2333-2

Synonyms

Definition

Gastrinomas are functioning gastrin-producing tumors of the neuroendocrine cells that are located in the duodenum, pancreas, and/or “gastrinoma triangle” and cause clinical Zollinger–Ellison syndrome (ZES).

Characteristics

Gastrinoma is a functioning neuroendocrine tumor of high malignant potential that follows an indolent clinical course. Neuroendocrine tumor cells release gastrin into the blood stream causing clinical symptoms, i.e., ZES.

Annual incidence of ZES in the US is estimated at 1 in 2.5 million people. It may occur in sporadic and familial forms. Twenty to 25 % of ZES cases are associated with familial multiple endocrine neoplasia type 1 (MEN1; ZES-MEN1) (endocrine-related cancers; carcinoid tumor; hereditary cancer syndromes). Sporadic ZES can develop at any age, but the majority of cases are diagnosed between the fourth and sixth decade of life (mean age 50.5 years). ZES-MEN1 patients have multiple...

Keywords

Multiple Endocrine Neoplasia Type Islet Cell Tumor Gastric Acid Output Acid Hypersecretion MEN1 Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
This is a preview of subscription content, log in to check access

References

  1. Debelenko L, Zhuang Z, Emmert-Buck MR et al (1997) Allelic deletions on chromosome 11q13 in multiple endocrine neoplasia type 1-associated and sporadic gastrinomas and pancreatic endocrine tumors. Cancer Res 57:2238–2243PubMedGoogle Scholar
  2. Jensen RT, Fraker DL (1994) Zollinger-Ellison syndrome: advances in treatment of gastric hypersecretion and gastrinoma. JAMA 18:1429–1435CrossRefGoogle Scholar
  3. Muscarella P, Melvin WS, Fisher WE et al (1998) Genetic alterations in gastrinomas and nonfunctioning pancreatic neuroendocrine tumors: an analysis of p16MTS1 tumor suppressor gene inactivation. Cancer Res 58:237–240PubMedGoogle Scholar
  4. Norton JA, Fraker DL, Alexander HR et al (1999) Surgery to cure the Zollinger-Ellison syndrome (see comments). N Engl J Med 341:635–644CrossRefPubMedGoogle Scholar
  5. Zhuang Z, Vortmeyer A, Pack S et al (1997) Somatic mutations of the MEN1 tumor suppressor gene in sporadic gastrinomas and insulinomas. Cancer Res 57:4682–4686PubMedGoogle Scholar

See Also

  1. (2012) Zollinger–Ellison syndrome. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3980. doi:10.1007/978-3-642-16483-5_6303Google Scholar
  2. (2012) CT. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, pp 1006–1007. doi:10.1007/978-3-642-16483-5_1398Google Scholar
  3. (2012) Homozygous deletion. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1729. doi:10.1007/978-3-642-16483-5_2807Google Scholar
  4. (2012) Hypermethylation. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1784. doi:10.1007/978-3-642-16483-5_2910Google Scholar
  5. (2012) MRI. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2382. doi:10.1007/978-3-642-16483-5_3854Google Scholar
  6. (2012) Octreotide. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2597. doi:10.1007/978-3-642-16483-5_4190Google Scholar
  7. (2012) Omeprazole. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2609. doi:10.1007/978-3-642-16483-5_4215Google Scholar
  8. (2012) P16INK4A. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2739. doi:10.1007/978-3-642-16483-5_4575Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg (outside the USA) 2016

Authors and Affiliations

  1. 1.National Cancer Institute, Division of Cancer Treatment and DiagnosisNational Institutes of HealthRockvilleUSA