Somatostatinoma

Last revised by Dr Jeremy Jones on 20 Sep 2021

Somatostatinomas are a rare type of neuroendocrine tumor

 They form up to ~1% of all gastroenteropancreatic endocrine neoplasms. 

The presentation can be variable. Patients with functional somatostatinoma may present with an "inhibitory syndrome" which is a triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhea. Other lesions can present with obstructive pancreatitis.

They typically occur in the pancreas but can also manifest in extrapancreatic organs (e.g. duodenum and ampulla of Vater) and can result in chronic obstructive pancreatitis.

They may be functional (i.e patients have high fasting somatostatin levels) or non-functional. Functional somatostatinomas can release excessive amounts of somatostatin 2.

As they share the radiographic features of other pancreatic endocrine tumors, please refer to this parenting article for a detailed discussion regarding these features.

Most somatostatinomas are malignant and are often associated with regional and/or portal metastases at the time of diagnosis 7.

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