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WHO Classification of Tumours
Somatostatinoma, malignant
Region of the ampulla of Vater


Definition

A somatostatinoma is a functionally active and usually malignant endocrine tumour with evidence of D-cell differentiation and clinical symptoms reflecting the diverse pathophysiologic effects of chronic inappropriate secretion of somatostatin (hypersomatostatinemia; somatostatinoma syndrome)
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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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Somatostatinomas account for between 1 to 2% of endocrine tumours of the gastroenteropancreaticohepatic (GEPH) axis and generally arise in adults. While some somatostatinomas are associated with neurofibromatosis type 1 (NF1) , multiple endocrine neoplasia (MEN1) and Von Hippel-Lindau syndromes , the etiology of their sporadic counterparts is unclear.

Commentary: Neuroendocrine tumours of the digestive system