Loading
4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
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).
How 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.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Resection by size and site; the parent page's pathways for metastatic disease; screening of NF1 patients for other tumours. | not mapped |