1/3. Duodenal somatostatinoma associated with diabetic ketoacidosis presumably caused by somatostatin-28 hypersecretion.CONTEXT: Extrapancreatic somatostatinoma is very rare and clinically distinguished from its pancreatic counterpart because somatostatinoma syndrome with mild diabetes is rare in extrapancreatic somatostatinoma because of poor secretion of somatostatin. Moreover, because somatostatin inhibits the secretion of insulin and glucagon simultaneously, true diabetic ketoacidosis (DKA) seldom ensues. PATIENT: A 23-yr-old woman presented with DKA and an abdominal mass. A computed tomography scan showed a huge, encapsulated mass in a duodenal submucous portion. A high circulating level of somatostatin was detected (67.2 pmol/liter; reference range, 0.6-7.3 pmol/liter). INTERVENTION: The tumor mass was successfully removed with Whipple's procedure, and the patient gradually recovered both clinically and biochemically. RESULTS: Immunohistochemical staining of the tumor tissue exhibited diffusely positive for somatostatin and somatostatin-28 but negative for insulin, glucagon, calcitonin, serotonin, and S-100. CONCLUSION: As far as we know, this is the first case report of gastrointestinal somatostatinoma associated with DKA.- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
2/3. Malignant somatostatinoma presenting with diabetic ketoacidosis.High circulating levels of somatostatin (SRIF) were detected in a patient with a metastatic tumour after development of diabetic ketoacidosis (DKA). fasting insulin and c-peptide levels were markedly suppressed, but plasma glucagon was not suppressed below normal. Progressive cachexia ensued; at autopsy a poorly differentiated non-small cell neuroendocrine carcinoma metastatic to liver was found. Small gallstones were noted. Electron microscopy of tumour tissue showed neurosecretory granules and tonofilament bundles. Immunohistochemical staining of tumour cells was diffusely positive for carcinoembryonic antigen, bombesin-like immunoreactivity, and calcitonin with focal immunoreactivity for SRIF, serotonin, neuron-specific enolase, chromogranin, and epithelial membrane antigen. Column chromatography of plasma and tumour extract revealed five or more peaks of material with SRIF-like immunoreactivity (SRIF-LI): predominantly SRIF-28 and intermediates in tumour extract, and SRIF-14 and an intermediate between SRIF-28 and SRIF-14 in plasma, DKA in this case of somatostatinoma syndrome may reflect differential effects of tumour production of larger molecular weight SRIF forms on insulin and glucagon secretion.- - - - - - - - - - ranking = 0.625keywords = somatostatinoma (Clic here for more details about this article) |
3/3. Pancreatic somatostatinoma: presentation with recurrent episodes of severe hyperglycaemia and ketoacidosis.A 47-year-old woman was admitted on four occasions over a four-year period with severe hyperglycaemia associated with marked ketoacidosis. She had weight loss with hepatomegaly and ultrasonography indicated a pancreatic tumour which was shown to be a somatostatinoma. Resection resulted in prolonged survival. The biochemical and morphological features of this rare tumour are presented, and an explanation for the unusual presentation of a somatostatinoma with episodes of ketoacidosis is given.- - - - - - - - - - ranking = 0.75keywords = somatostatinoma (Clic here for more details about this article) |