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WHO Classification of Tumours
Gastrinoma, malignant
Pancreas


Definition

A gastrinoma is a functionally active and usually malignant endocrine tumour with clinical symptoms due to inappropriate secretion of gastrin (Zollinger Ellison syndrome; ZES)
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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

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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

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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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Commentary: Neuroendocrine tumours of the digestive system