Pancreatic Somatostatinoma Presenting With Hypoglycaemia

Abstract

A 33-year-old woman with a 2 month history of vague ill health was admitted to hospital in hypoglycaemic coma. Preoperative investigation suggested malignant insulinoma as the probable cause of illness, but immunohistological examination of the tumour showed it to consist mainly of somatostatin-containing cells but sparse insulin-secreting cells were also present. Plasma immunoreactive somatostatin levels were from fifty to 200 times the upper limit of normal and rose in response to arginine and fell during diazoxide infusion. The hypoglycaemia was unusually sensitive to the hyperglycaemic effects of diazoxide and chlorothiazide and, with 5-fluorouracil as the only specific anti-tumour agent, there has been clinical, biochemical and radiological evidence of tumour regression.

MANAGEMENT OF ENDOCRINE DISEASE: A clinical update on tumor-induced hypoglycemia

“…The most common symptoms are abdominal pain and weight loss and, in some instances (w10%) they produce somatostatinoma syndrome (diabetes mellitus, cholelithiasis, and diarrhea with steatorrhea) due to excessive secretion of somatostatin, a hormone that inhibits gastrointestinal motility and secretion as well as the release of many hormones including insulin and glucagon. Somatostatinoma-induced hypoglycemia is uncommon with isolated cases reported to date (81,82,83,84). In three out of four reported cases, the tumor was located in the pancreas (82,83,84) and only one in the ovary (81).…”

“…Somatostatinoma-induced hypoglycemia is uncommon with isolated cases reported to date (81,82,83,84). In three out of four reported cases, the tumor was located in the pancreas (82,83,84) and only one in the ovary (81). The mechanism of somatostatinomainduced hypoglycemia is not clear.…”

Endokrines System und Schock: Therapeutische Perspektiven

“…Bedingt durch die gegenregulatorische Adrenalinausschüttung (CHRISTENSEN 1979;GERICH et al 1979;CRYER 1980;SANTEUSANIO 1981) RAMSON et al 1966;CORRALL et al 1981) oder durch spontanen Somatostatinexzeß WRIGHT et al 1980) blockiert ist oder wenn die gegenregulatorischen Hormone aus anderen Gründen nicht adäquat ausgeschüttet wer den (CHRISTENSEN 1979;GRIES etal. 1980;HILSTED 1980;HILSTED etal.…”

Diffuse somatostatin-immunoreactive D-cell hyperplasia in the stomach and duodenum

“…Like gastrinomas, the tumors were mostly malignant and with few exceptions were predominantly located in the pancreas: two tumors in the duodenum (21,22) and one in the jejunum (23). The clinical symptoms were heterogeneous and depended on the serum somatostatin levels and probably on the composition of the different molecular weight forms of the circulating hormones (24,25), the hormonal heterogeneity of some somatostatinomas, and probably on the vary ing degrees of target organ resistance to the increased hormone levels (26).…”