Somatostatinoma
Citation, DOI, disclosures and article data
At the time the article was created Yuranga Weerakkody had no recorded disclosures.
View Yuranga Weerakkody's current disclosuresAt the time the article was last revised Joshua Yap had no financial relationships to ineligible companies to disclose.
View Joshua Yap's current disclosures- Somatostatinomas
Somatostatinomas are a rare type of neuroendocrine tumor.
On this page:
Epidemiology
They form up to ~1% of all gastroenteropancreatic endocrine neoplasms.
Associations
Clinical presentation
The presentation can be variable. Patients with functional somatostatinoma may present with an "inhibitory syndrome" which is a triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhea. Other lesions can present with obstructive pancreatitis.
Pathology
They typically occur in the pancreas but can also manifest in extrapancreatic organs (e.g. duodenum and ampulla of Vater) and can result in chronic obstructive pancreatitis.
They may be functional (i.e. patients have high fasting somatostatin levels) or non-functional. Functional somatostatinomas can release excessive amounts of somatostatin 2.
Radiographic features
As they share the radiographic features of other pancreatic neuroendocrine tumors, please refer to this parenting article for a detailed discussion regarding these features.
Treatment and prognosis
Most somatostatinomas are malignant and are often associated with regional and/or portal metastases at the time of diagnosis 7.
See also
References
- 1. Tjon A Tham RT, Jansen JB, Falke TH et-al. Imaging features of somatostatinoma: MR, CT, US, and angiography. J Comput Assist Tomogr. 1994;18 (3): 427-31. Pubmed citation
- 2. Nesi G, Marcucci T, Rubio CA et-al. Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J. Gastroenterol. Hepatol. 2008;23 (4): 521-6. doi:10.1111/j.1440-1746.2007.05053.x - Pubmed citation
- 3. Soga J, Yakuwa Y. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J. Exp. Clin. Cancer Res. 1999;18 (1): 13-22. Pubmed citation
- 4. Garbrecht N, Anlauf M, Schmitt A et-al. Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocr. Relat. Cancer. 2008;15 (1): 229-41. doi:10.1677/ERC-07-0157 - Pubmed citation
- 5. Roberts L, Dunnick NR, Foster WL et-al. Somatostatinoma of the endocrine pancreas: CT findings. J Comput Assist Tomogr. 1984;8 (5): 1015-8. Pubmed citation
- 6. Yu RS, Chen Y, Wang LH et-al. A large functional somatostatinoma in the pancreatic tail: atypical CT appearances. Turk J Gastroenterol. 2010;20 (4): 291-4. Pubmed citation
- 7. House MG, Yeo CJ, Schulick RD. Periampullary pancreatic somatostatinoma. Ann. Surg. Oncol. 2003;9 (9): 869-74. Pubmed citation
- 8. Skucas J. Advanced Imaging of the Abdomen. Springer. ISBN:1852339926. Read it at Google Books - Find it at Amazon
- 9. Tanaka S, Yamasaki S, Matsushita H et-al. Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis. Pathol. Int. 2000;50 (2): 146-52. Pubmed citation
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