Duodenal somatostatinoma associated with diabetic ketoacidosis presumably caused by somatostatin-28 hypersecretion.
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.
Kim
Dol Mi
DM
Department of Endocrinology, Yonsei University, College of Medicine, Seoul 120-752, Korea.
Ahn
Chul Woo
CW
Kim
Kyung Rae
KR
Hong
Soon Won
SW
Nam
Moon Suk
MS
Cha
Bong Soo
BS
Lim
Sung Kil
SK
Lee
Hyun Chul
HC
Lee
Eun Jig
EJ
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
D013004:Somatostatin; D053561:Somatostatin-28; D004247:DNA