8153/3 Gastrin cell tumor, malignant


Related terms

Gastrinoma, malignant

Definitions

Small intestine
ICD-O-3 topography code: C17
ICD10: C17
           

Gastrinoma is a neuroendocrine tumour (NET) characterized by the production of gastrin. Approximately 15% of gastrinomas cause Zollinger-Ellison syndrome (ZES). They occur earlier in life than their non-functioning counterparts and carry a higher risk of metastasis 1
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

A gastrinoma is a functionally active and usually malignant endocrine tumour with clinical symptoms due to inappropriate secretion of gastrin (Zollinger Ellison syndrome; ZES)
2
 
Solcia E, Capella C, Kloppel G
Tumors of the Pancreas
3rd ed.
Armed Forces Institute of Pathology: Washington 1997



. Two types of gastrinomas can be distinguished, sporadic, non-familial gastrinoma with ZES (≈80% of cases), and familial gastrinoma with ZES in the setting of MEN 1 (≈20%).

Gastrinomas are relatively common functionally active endocrine tumour of the pancreas accounting for about 20% of cases, second in frequency only to insulinomas
3
 
Kloppel G, Heitz PU
Pancreatic pathology
Churchill Livingstone: Edinburgh/New York 1984



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The etiology and pathogenesis of sporadic gastrinomas are unknown.
Most patients with gastrinomas suffer from typical duodenal ulcer at presentation.
Gastrinomas show a high risk of malignant behaviour independent of size and should therefore be classified as tumours with uncertain behaviour or as well-differentiated endocrine carcinomas when gross invasion and/or metastases are present
4
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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Stomach
ICD-O-3 topography code: C16
ICD10: C16.9
           

Most neuroendocrine neoplasms of the stomach are neuroendocrine tumours (NETs) ? well-differentiated, nonfunctioning enterochromaffin-like (ECL) cell carcinoids (ECL cell NETs) that mainly produce histamine and histidine decarboxylase. However, minor cell subpopulations expressing serotonin, ghrelin, gastrin, somatostatin, pancreatic polypeptide (PP), or α-human chorionic gonadotrophin (α-hCG) have been detected
5
Click to access Pubmed
Papotti M, Cassoni P, Volante M, Deghenghi R, Muccioli G, Ghigo E (2001)
Ghrelin-producing endocrine tumors of the stomach and intestine.
J Clin Endocrinol Metab 86: 5052-9



6
Click to access Pubmed
Rindi G, Luinetti O, Cornaggia M, Capella C, Solcia E (1993)
Three subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine carcinoma: a clinicopathologic study.
Gastroenterology 104: 994-1006



7
Click to access Pubmed
Rindi G, Savio A, Torsello A, Zoli M, Locatelli V, Cocchi D, Paolotti D, Solcia E (2002)
Ghrelin expression in gut endocrine growths.
Histochem Cell Biol 117: 521-5



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Gastrin producing NET (gastrinoma)of the stomach is rare. NETs that produce gastrin may present in association with Zollinger-Ellison syndrome (ZES) due to overproduction of gastrin and may thus be defined as gastrinoma (more frequently occurring in the duodenum). It should be noted that immunohistochemical demonstration of gastrin in the absence of a syndromic condition does not merit use of the term gastrinoma. Well-differentiated gastrin-producing NETs are small, mucosal-submucosal nodules, found incidentally at endoscopy or in a gastrectomy specimen, more frequently in proximity of the pylorus, either on its gastric or, especially, its duodenal slope. They may show a characteristic thin, trabecular? gyriform pattern or a solid nest pattern. The cells are uniform with scanty cytoplasm and show predominant immunoreactivity for gastrin
8
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Small intestine
ICD-O-3 topography code: C17
ICD10: C17