Published date: Sep 24 2018

Journal Title: Sudan Journal of Medical Sciences

Issue title: Sudan JMS: Volume 13 (2018), Issue No. 3

Pages: 187–195

DOI: 10.18502/sjms.v13i3.2956

Ayman Fisal Ahmed Foad - a.foad@ut.edu.sa

Hyder Osman Mirghani

Somatostatinoma is a neuroendocrine tumor; its incidence in the duodenum around the ampulla of Vater is rare, and it is often not associated with secretory manifestation. We report a case of a female with an ampullary (Vater) tumor displaying neuroendocrine nuclear features, psammoma bodies, and the positivity for immunohistochemical panel of neuroendocrine tumors. Our patient presented with some features compatible with somatostatin secretion associated syndrome; albeit with normal serum levels of the hormone. Initial attempt of complete resection failed, and the involved margins were revised with a subsequent surgery, and the patient showed an uneventful course on follow-up for 2 years.

[1] Bosman, F. (2010). WHO Classification of Tumours of the Digestive System. Lyon: International Agency for Research on Cancer.

[2] Medscape. (2018). Somatostatinomas: Background, Pathophysiology, Epidemiology. Retrieved from http://emedicine.medscape.com/article/182841-clinical (accessed on September 4, 2018).

[3] Cooper, H. S. (2015). Intestinal neoplasms, in S. E. Mills and S. S. Sternberg. Sternberg’s Diagnostic Surgical Pathology, p. 1552. Philadelphia, PA: Lippincott Williams et Wilkins.

[4] Medscape. (2018). Somatostatin: Reference Range, Background/Interpretation, Collection and Panels. Retrieved from https://emedicine.medscape.com/article/ 2089466-overview (accessed on September 4, 2018).

[5] Pathologyoutlines.com. (2018). TNM Staging. Retrieved from: http://www. pathologyoutlines.com/topic/ampullatnmamp.html (accessed on September 4, 2018).

[6] Kaneko, H., Yanahira, N., Ito, S., et al. (1979). Somatostatinoma of the duodenum. Cancer, vol. 44, pp. 2273–2279.

[7] Yamamoto, R., Kato, S., Maru, T., et al. (2016). The coexistence of somatostatinoma and gastrointestinal stromal tumor in the duodenum of a patient with Von Recklinghausen’s Disease. Internal Medicine, vol. 55, no. 6, pp. 617–622. DOI:10.2169/internalmedicine.55.5761

[8] Kim, T. U., Kim, S., Lee, J. W., et al. (2008). Ampulla of Vater: Comprehensive anatomy, MR imaging of pathologic conditions, and correlation with endoscopy. European Journal of Radiology, vol. 66, no. 1, pp. 48–64.
DOI:10.1016/j.ejrad.2007.04.005

[9] Kainuma, O., Ito, Y., Taniguchi, T., et al. (1996). Ampullary somatostatinoma in a patient with von Recklinghausen’s disease. Journal of Gastroenterology, vol. 31, pp. 460–464.

[10] Karasawa, Y., Sakaguchi, M., Minami, S., et al. (2001). Duodenal somatostatinoma and erythrocytosis in a patient with von Hippel-Lindau disease type 2. Internal Medicine, vol. 40, pp. 38–43.

[11] Sreenarasimhaiah, J., Armstrong, L. A., Tang, S.-J., et al. (2009). Pancreatic somatostatinoma and tuberous sclerosis: Case report of an exceedingly rare association. Gastrointestinal Endoscopy, vol. 69, no. 2, pp. 379–381.

[12] NHS Choices. Department of Health. Tuberous sclerosis. Retrieved from http:// www.nhs.uk/conditions/Tuberous sclerosis/pages/introduction.aspx (accessed onJanuary 8, 2017).

[13] Marini, F., Falchetti, A., Luzi, E., et al. (2009). Multiple Endocrine Neoplasia Type 1 (MEN1) Syndrome, in D. L. Riegert-Johnson, L. A. Boardman, T. Hefferon, et al. (eds.) Cancer Syndromes. Bethesda (MD): National Center for Biotechnology Information (US). Retrieved from: https://www.ncbi.nlm.nih.gov/books/NBK7029/ (accessed
on July 18, 2008 [Updated August 9, 2008]).

[14] Garbrecht, N., Anlauf, M., Schmitt, A., et al. (2008). Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: Incidence, types, biological behavior, association with inherited syndromes, and functional activity. EndocrineRelated Cancer, vol. 15, pp. 229–241.

[15] Vinik, A., Feliberti, E., Perry, R. R. (2000). Somatostatinoma, in L. J. De Groot, G. Chrousos, K. Dungan, et al. (eds.) Endotext. South Dartmouth (MA): MDText.com, Inc. Retrieved from: https://www.ncbi.nlm.nih.gov/books/NBK279034/ (accessed on November 28, 2013).

[16] International Diabetes Federation, Sudan. (n.d.). Retrieved from http://www.idf. org/membership/mena/sudan (accessed on January 18, 2017).

[17] Duodenum (Psammomatous) Somatostatinoma. (n.d.). Retrieved from http://surgpathcriteria.stanford.edu/gitumors/duodenum-somatostatinomapsammomatous/printable.html ( January 18, 2017).

[18] Vinik, A., Feliberti, E., and Perry, R. R. (2000). Somatostatinoma, in L. J. De Groot, G. Chrousos, K. Dungan, et al. (eds.) Endotext. South Dartmouth (MA): MDText.com, Inc. Retrieved from: https://www.ncbi.nlm.nih.gov/books/NBK279034/ (accessed on November 28, 2013).