• James M. L. WilliamsonEmail author


Somatostatinomas are rare neuroendocrine tumours (NETs) that arise in the pancreas or duodenum. They are the fifth most common pancreatoduodenal NET (after insulinomas, non-functioning tumours, gastrinomas and VIPomas), with an incidence of 1 in 40 million [1–3]. They tend to be non-functioning, although they are rarely active and can produce a spectrum of symptoms called ‘somatostatinoma syndrome’ [4–6]. A degree of discord exists regarding the functional component of a somatostatinoma; some clinicians argue that a ‘syndrome’ of clinical symptoms must be present for the diagnosis of somatostatinoma and others that the diagnosis is purely immunohistochemical [7]. As most of these tumours are biochemically inert, they are either detected incidentally or by causing mass-effect pressure symptoms, and most will have metastasised by the time of diagnosis [6, 8, 9].


Hepatic Metastasis Peptide Receptor Radionuclide Therapy Operative Resection Hepatic Artery Embolisation Rare Neuroendocrine Tumour 
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© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Hepato-pancreato-biliary surgeryBristol Royal InfirmaryBristolUK

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