This Article Full Text Full Text (PDF) Supplemental Table Submit a related Letter to the Editor Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Kim, D. M. Articles by Lee, E. J. Search for Related Content PubMed PubMed Citation Articles by Kim, D. M. Articles by Lee, E. J. Related Collections Diabetes and Insulin Endocrine Oncology Metabolism

Duodenal Somatostatinoma Associated with Diabetic Ketoacidosis Presumably Caused by Somatostatin-28 Hypersecretion

Departments of Endocrinology (D.M.K., C.W.A., K.R.K., B.S.C., S.K.L., H.C.L.) and Pathology (S.W.H.), Yonsei University, College of Medicine, Seoul 120-752, Korea; Department of Endocrinology (M.S.N.), Inha University College of Medicine, Incheon 400-711, Korea; and Department of Endocrinology, Metabolism, and Molecular Medicine (E.J.L.), Northwestern University, Feinberg School of Medicine, Chicago, Illinois 60611

Address all correspondence and requests for reprints to: Chul Woo Ahn, M.D., Yongdong Severance Hospital, Yonsei University, College of Medicine, 146-92 Dogok-Dong, Kangnam-Ku, Seoul, Korea. E-mail: acw{at}yumc.yonsei.ac.kr.

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.