title: Duodenal somatostatinoma associated with diabetic ketoacidosis presumably caused by somatostatin-28 hypersecretion.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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).
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  As far as we know, this is the first case report of gastrointestinal somatostatinoma associated with DKA.
authors.affiliation: Department of Endocrinology, Yonsei University, College of Medicine, Seoul 120-752, Korea.
authors.forename: Dol Mi
authors.initials: DM
authors.lastname: Kim
authors.forename: Chul Woo
authors.initials: CW
authors.lastname: Ahn
authors.forename: Kyung Rae
authors.initials: KR
authors.lastname: Kim
authors.forename: Soon Won
authors.initials: SW
authors.lastname: Hong
authors.forename: Moon Suk
authors.initials: MS
authors.lastname: Nam
authors.forename: Bong Soo
authors.initials: BS
authors.lastname: Cha
authors.forename: Sung Kil
authors.initials: SK
authors.lastname: Lim
authors.forename: Hyun Chul
authors.initials: HC
authors.lastname: Lee
authors.forename: Eun Jig
authors.initials: EJ
authors.lastname: Lee
mesh_terms: D000328:Adult; D004247:DNA / Q000032:analysis; D016883:Diabetic Ketoacidosis / Q000150:complications*; D004379:Duodenal Neoplasms / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D013004:Somatostatin / Q000032:analysis / Q000378:metabolism*; D053561:Somatostatin-28; D013005:Somatostatinoma / Q000209:etiology* / Q000378:metabolism / Q000473:pathology
chemical_list: D013004:Somatostatin; D053561:Somatostatin-28; D004247:DNA