Duodenal somatostatinoma associated with diabetic ketoacidosis presumably caused by somatostatin-28 hypersecretion.

Abstract

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.

• Authors: Dol Mi Kim, Chul Woo Ahn, Kyung Rae Kim, Soon Won Hong, Moon Suk Nam, Bong Soo Cha, Sung Kil Lim, Hyun Chul Lee, Eun Jig Lee
• Journal: The Journal of clinical endocrinology and metabolism (J Clin Endocrinol Metab) Vol. 90 Issue 11 Pg. 6310-5 (Nov 2005) ISSN: 0021-972X [Print] United States
• PMID: 16105971 (Publication Type: Case Reports, Journal Article)

Chemical References

• Somatostatin
• Somatostatin-28
• DNA

Topics

• Adult
• DNA (analysis)
• Diabetic Ketoacidosis (complications)
• Duodenal Neoplasms (etiology, metabolism, pathology)
• Female
• Humans
• Immunohistochemistry
• Somatostatin (analysis, metabolism)
• Somatostatin-28
• Somatostatinoma (etiology, metabolism, pathology)