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Pancreatic Somatostatinoma

المؤلف:  Wass, J. A. H., Arlt, W., & Semple, R. K. (Eds.).

المصدر:  Oxford Textbook of Endocrinology and Diabetes

الجزء والصفحة:  3rd edition , p1030

2026-09-01

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Somatostatinoma syndrome was first described in 1977. Over 100 such cases have now been reported with features as in Box 1. The syndrome consists of cholelithiasis, the cause of which is multi factorial, including suppression of CCK production which results in impaired gallbladder contractility. High levels of somatostatin also inhibit bowel transit, which alters bowel flora, thus increasing bile acid reabsorption and this is associated with super saturated bile. Mild diabetes occurs and has often been present for many years before diagnosis. It is probably due to suppression of insulin secretion. Diarrhoea and steatorrhoea also occur and relate to the inhibition of pancreatic exocrine function. Hypochlorhydria relates to the inhibition of gastric acid secretion and gastrin. Anaemia, abdominal pain, and weight loss are also present and are non- specific. They are probably related to the size of the tumour, which is usually large, and also to the fact that it is malignant. Those tumours are often diagnosed late and distant metastases may be present in lymph nodes, liver, or bone (55% are in the head of the pancreas).

Box1. Features of pancreatic somatostatinoma

Plasma and tissue levels of somatostatin are elevated and levels are higher in pancreatic as opposed to duodenal somatostatinomas. These somatostatin- secreting cells often also secrete adrenocorticotrophic hormone (ACTH), calcitonin, insulin, or some other peptides. This means that Cushing’s syndrome, flushing, or hypo glycaemia (if there is cosecretion of insulin) may be present.