Somatostatinoma syndrome. Clinical, morphological and metabolic features and therapeutic aspects

Abstract

A case of somatostatinoma syndrome in a 30-year-old woman is presented. Basal levels of growth hormone and of pancreatic and gastric hormones were reduced and the response of growth hormone, insulin and C-peptide to stimuli such as arginine, glucose, glibenclamide and calcium was virtually abolished. Similarly, gastric acid secretion, pancreatic exocrine function and intestinal absorption were significantly reduced. On the other hand, basal and stimulated levels of adrenocorticotropic hormone (ACTH), luteinizing hormone (LH), follicle-stimulating hormone (FSH) and thyroid-stimulating hormone (TSH) were within the normal range. Plasma somatostatin-like immunoreactivity was increased to 600-2,000 pg/ml (normal: 88-140 pg/ml). Immunocytochemical studies demonstrated the presence of somatostatin immunoreactive material in the primary tumour in the head of the pancreas and in the liver metastases. In spite of two courses of chemotherapy with streptozotocin and 5-fluorouracil the patient died due to liver failure 5 months after the first admission to hospital.

A Gastric Neuroendocrine Carcinoma Expressing Somatostatin in a Bearded Dragon (Pogona Vitticeps)

“…26 In humans, somatostatin has been shown to suppress pancreatic secretion of insulin, 1 glucagons, 10 and pancreatic polypeptides. 22 Similarly, in the intestinal tract, somatostatin inhibits gastrin release, gastric acid secretion, gastric motility, intestinal motility, intestinal absorption, and gallbladder contraction. [3][4][5] This leads to a particular syndrome characterized by diabetes mellitus, hypochlorhydria, pancreatic exocrine insufficiency, and gallstones.…”

Chronic Refractory Diarrhoea: A Manifestation of Endocrine Disorders

“…Diarrhoea was also reported in two recent case reports [110,111]. Although the precise mechanism of diarrhoea in somatostatinoma is unknown, high circulating levels of soma tostatin have been shown to cause a reduction of the small intestinal absorption of water, electrolytes, glucose and amino acids, and ste atorrhoea appears to be due to impaired exo crine secretion of the pancreas [19,34,112], Although somatostatin powerfully inhibits the release of many hormones and also the action of many hormones it appears that there is a considerable potential for 'escape' from the chronically elevated levels found in soma tostatinoma [32], Thus early diagnosis of somatostinoma is difficult and diagnosis is usually made late in their clinical courses. The triad of dyspepsia, diabetes mellitus and cho lelithiasis should arouse suspicion [107], A high circulating level of plasma somatostatin is present in most cases [107], In some cases provocative tests like calcium-pentagastrin and tolbutamide test are required for docu mentation of enhanced release of somatosta tin from the tumour [113,114], Like other pancreatic tumours, ultrasonography, com puterized axial tomography, angiography, percutaneous transhepatic portal or pancreat ic venous sampling for somatostatin estima tion may be required for localization of the tumour [107], At present there is insufficient experience to recommend an optimal method of treatment.…”

“…The triad of dyspepsia, diabetes mellitus and cho lelithiasis should arouse suspicion [107], A high circulating level of plasma somatostatin is present in most cases [107], In some cases provocative tests like calcium-pentagastrin and tolbutamide test are required for docu mentation of enhanced release of somatosta tin from the tumour [113,114], Like other pancreatic tumours, ultrasonography, com puterized axial tomography, angiography, percutaneous transhepatic portal or pancreat ic venous sampling for somatostatin estima tion may be required for localization of the tumour [107], At present there is insufficient experience to recommend an optimal method of treatment. Both surgery and chemotherapy are used without very encouraging results [107,112], However, after complete surgical removal of rare benign somatostatinomas the prognosis of patients appears to be good [115,116],…”

A Metastatic Endocrine-Neurogenic Tumor of the Ampulla of Vater with Multiple Endocrine Immunoreaction Malignant Paraganglioma?

“…The patient had no history of a biliary ob structive disease or pancreatitis and no signs of a somatostatinoma, such as gallstones, steatorrhea, di arrhea. or malabsorption syndrome [14]. On admis sion he had no gastrointestinal complaints and was feeling well.…”