{"entity":{"id":"somatostatinoma","kind":"cancer","name":"Somatostatinoma","aka":["Somatostatin-secreting neuroendocrine tumour","Duodenal somatostatinoma","Delta-cell tumour","Inhibitory syndrome tumour"],"tldr":"Somatostatinoma is one of the rarest neuroendocrine tumours, arising in the pancreas or the duodenum and secreting somatostatin, a hormone that switches off digestion, which can cause mild diabetes, gallstones and fatty diarrhoea. Duodenal cases are often linked to neurofibromatosis type 1 and rarely cause symptoms. Surgery is the main treatment and the outlook depends on site and grade.","summary":"Somatostatinoma is a rare neuroendocrine tumour of delta cells, about 1 percent of gastroenteropancreatic endocrine neoplasms, most often in the duodenum followed by the pancreas; it may be sporadic or associated with neurofibromatosis type 1, MEN1 and von Hippel-Lindau disease, and functional tumours cause the inhibitory syndrome of mild diabetes, cholelithiasis and diarrhoea or steatorrhoea by suppressing gallbladder motility and endocrine and exocrine secretion, while non-functional tumours are asymptomatic or obstructive (J Gastroenterol Hepatol 2008). The incidence is about 1 in 40 million; duodenal and periampullary tumours cause jaundice or pancreatitis, and the NF1 association reflects RAS-MAPK pathway dysfunction, with the Pacak-Zhuang syndrome (EPAS1) a rarer genetic setting; surgery is central where feasible and prognosis depends on location and WHO grade (Experimental and Therapeutic Medicine 2020).\n\nHow it differs from its parent: it is the functioning tumour whose hormone inhibits rather than stimulates, so its syndrome is subtle and most cases are found by obstruction or incidentally; duodenal tumours are usually small, often psammomatous and syndromic, whereas pancreatic tumours are larger and more often metastatic.\n\nHow common: about 1 in 40 million (Experimental and Therapeutic Medicine 2020).\n\nTreatment: resection (pancreatoduodenectomy or local excision by size and site); the parent page's pathways (somatostatin analogues, everolimus, sunitinib, peptide receptor radionuclide therapy) for metastatic disease, with grade guiding the choice; NF1 patients are screened for other tumours.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Somatostatinoma","links":[{"label":"NCI PDQ: pancreatic neuroendocrine (islet cell) tumour treatment","url":"https://www.cancer.gov/types/pancreatic/neuroendocrine-tumors/treatment"},{"label":"J Gastroenterol Hepatol 2008: somatostatinoma, three cases and literature review","url":"https://doi.org/10.1111/j.1440-1746.2007.05053.x"},{"label":"Experimental and Therapeutic Medicine 2020: somatostatinoma beyond neurofibromatosis type 1 (review)","url":"https://doi.org/10.3892/etm.2020.8965"}],"tags":["subtype-page","wave4","rare"],"related":["pancreatic-net","glucagonoma","vipoma","small-intestinal-net","men1-syndrome"],"cancers":[],"sections":[],"technologies":["prrt"],"targets":["nf1"],"drugs":["everolimus","sunitinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"endocrine","burden":"About 1 percent of gastroenteropancreatic endocrine neoplasms, with roughly 200 published cases by 2008 (J Gastroenterol Hepatol 2008); an incidence of about 1 in 40 million people (Experimental and Therapeutic Medicine 2020).","subtypes":["Duodenal somatostatinoma (often NF1-associated, psammomatous, small)","Pancreatic somatostatinoma (larger, more often metastatic)","Functional somatostatinoma with the inhibitory syndrome","Non-functional somatostatin-expressing neuroendocrine tumour (incidental or obstructive)"],"biomarkers":["Plasma somatostatin","NF1 status; MEN1 and VHL in syndromic cases","WHO grade (Ki-67) and site","Somatostatin receptor imaging"],"standardOfCare":[{"setting":"All cases","approach":"Resection by size and site; the parent page's pathways for metastatic disease; screening of NF1 patients for other tumours.","refs":["pancreatic-net","everolimus","sunitinib","prrt"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"pancreatic-net"},"route":"/cancers/somatostatinoma/","neighbours":{"cancer":[{"id":"glucagonoma","kind":"cancer","name":"Glucagonoma","route":"/cancers/glucagonoma/"},{"id":"men1-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 1 (MEN1)","route":"/cancers/men1-syndrome/"},{"id":"pancreatic-net","kind":"cancer","name":"Pancreatic neuroendocrine tumours","route":"/cancers/pancreatic-net/"},{"id":"small-intestinal-net","kind":"cancer","name":"Small intestinal neuroendocrine tumours","route":"/cancers/small-intestinal-net/"},{"id":"vipoma","kind":"cancer","name":"VIPoma","route":"/cancers/vipoma/"}],"technology":[{"id":"prrt","kind":"technology","name":"Peptide receptor radionuclide therapy (PRRT)","route":"/technologies/prrt/"}],"target":[{"id":"nf1","kind":"target","name":"NF1 (neurofibromin)","route":"/targets/nf1/"}],"drug":[{"id":"everolimus","kind":"drug","name":"Everolimus","route":"/drugs/everolimus/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"}]}}