Journal of Zhejiang University SCIENCE B
ISSN 1673-1581(Print), 1862-1783(Online), Monthly

2010   Vol. 11   No. 1   p. 22~26

On-line Access Date:   Jan. 1, 2010
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Pancreatic somatostatinoma with obscure inhibitory syndrome and mixed pathological pattern

Bo ZHANG1, Qiu-ping XIE1, Shun-liang GAO1, Yan-biao FU2, Yu-lian WU†‡1

(1Department of Surgery, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China)
(2Department of Pathology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China)
Corresponding Author
Received June 12, 2009; revision accepted Oct. 13, 2009; Crosschecked Dec. 2, 2009

Abstract: Somatostatinoma is a very rare neuroendocrine tumor that originates from D cells and accounts for less than 1% of all gastrointestinal endocrine tumors. The duodenum is the most frequent site for this tumor, followed by the pancreas. We here describe a 46-year-old Chinese woman who developed pancreatic somatostatinoma presenting with the characteristic “inhibitory” syndrome, but the symptoms were obscure and seemingly uncorrelated. This case is also unique for its large tumor size and mixed pathological pattern. Distal pancreatectomy was performed, and the patient has remained well since operation. As the syndromes of somatostatinoma may be obscure and atypical, clinicians should review all clinical findings to obtain an accurate diagnosis. Aggressive surgery is preferred to improve the survival.

Key words: Neuroendocrine tumor, Somatostatinoma, Somatostatinoma syndrome, Pancreatic hormone-producing tumor, Pancreatectomy
doi:10.1631/jzus.B0900166             CLC number: R73


[1] Anene, C., Thompson, J.S., Saigh, J., Badakhsh, S., Ecklund, R.E., 1995. Somatostatinoma: atypical presentation of a rare pancreatic tumor. Am. J. Gastroenterol., 90(5): 819-821.

[2] Barahona-Garrido, J., Aguirre-Gutierrez, R., Gutierrez-Manjarrez, J.I., Tellez-Avila, F.I., Lopez-Arce, G., Fomperoza-Torres, A., Criales, S., Sanchez-Cortes, E., Sarti, H.M., Yamamoto-Furusho, J.K., 2009. Association of GIST and somatostatinoma in a patient with type-1 neurofibromatosis: is there a common pathway? Am. J. Gastroenterol., 104(3):797-799.

[3] Cao, X.P., Liu, Y.Y., Xiao, H.P., Li, Y.B., Wang, L.T., Xiao, P., 2009. Pancreatic somatostatinoma characterized by extreme hypoglycemia. Chin. Med. J. (Engl.), 122(14): 1709-1712.

[4] House, M.G., Yeo, C.J., Schulick, R.D., 2002. Periampullary pancreatic somatostatinoma. Ann. Surg. Oncol., 9(9):869-874.

[5] Janson, E.T., 2005. Somatostatin analogs in the treatment of neuroendocrine gastroenteropancreatic and intrathoracic tumors. J. Endocrinol. Invest., 28(11 Suppl. International): 137-140.

[6] Klöppel, G., Perren, A., Heitz, P.U., 2004. The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification. Ann. N. Y. Acad. Sci., 1014(1):13-27.

[7] Konomi, K., Chijiiwa, K., Katsuta, T., Yamaguchi, K., 1990. Pancreatic somatostatinoma: a case report and review of the literature. J. Surg. Oncol., 43(4):259-265.

[8] Larsson, L.I., Hirsch, M.A., Holst, J.J., Ingemansson, S., Kuhl, C., Jensen, S.L., Lundqvist, G., Rehfeld, J.F., Schwartz, T.W., 1977. Pancreatic somatostatinoma. Clinical features and physiological implications. Lancet, 309(8013): 666-668.

[9] Levy-Bohbot, N., Merle, C., Goudet, P., Delemer, B., Calender, A., Jolly, D., Thiefin, G., Cadiot, G., 2004. Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas: study from the GTE (Groupe des Tumeurs Endocrines) registry. Gastroenterol. Clin. Biol., 28(11):1075-1081.

[10] Maki, M., Kaneko, Y., Ohta, Y., Nakamura, T., Machinami, R., Kurokawa, K., 1995. Somatostatinoma of the pancreas associated with von Hippel-Lindau disease. Intern. Med., 34(7):661-665.

[11] Marakis, G., Ballas, K., Rafailidis, S., Alatsakis, M., Patsiaoura, K., Sakadamis, A., 2005. Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis: a case report. Pancreatology, 5(2-3):295-299.

[12] Moayedoddin, B., Booya, F., Wermers, R.A., Lloyd, R.V., Rubin, J., Thompson, G.B., Fatourechi, V., 2006. Spectrum of malignant somatostatin-producing neuroendocrine tumors. Endocr. Pract., 12(4):394-400.

[13] Mozell, E., Stenzel, P., Woltering, E.A., Rosch, J., O'Dorisio, T.M., 1990. Functional endocrine tumors of the pancreas: clinical presentation, diagnosis, and treatment. Curr. Probl. Surg., 27(6):309-386.

[14] Nesi, G., Marcucci, T., Rubio, C.A., Brandi, M.L., Tonelli, F., 2008. Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J. Gastroenterol. Hepatol., 23(4):521-526.

[15] Sakazaki, S., Umeyama, K., Nakagawa, H., Hashimoto, H., Kamino, K., Mitsuhashi, T., Yamaguchi, K., 1983. Pancreatic somatostatinoma. Am. J. Surg., 146(5):674-679.

[16] Sato, T., Konishi, K., Kimura, H., Maeda, K., Yabushita, K., Tsuji, M., Demachi, H., Miwa, A., 2000. Strategy for pancreatic endocrine tumors. Hepatogastroenterology, 47(32):537-539.

[17] Soga, J., Yakuwa, Y., 1999. Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. J. Exp. Clin. Cancer Res., 18(1):13-22.

[18] Stelow, E.B., Woon, C., Pambuccian, S.E., Thrall, M., Stanley, M.W., Lai, R., Mallery, S., Gulbahce, H.E., 2005. Fine-needle aspiration cytology of pancreatic somatostatinoma: the importance of immunohistochemistry for the cytologic diagnosis of pancreatic endocrine neoplasms. Diagn. Cytopathol., 33(2):100-105.

[19] Suzuki, H., Kuwano, H., Masuda, N., Hashimoto, S., Kanoh, K., Nomoto, K., Shimura, T., Katoh, H., 2008. Diagnostic usefulness of FDG-PET for malignant somatostatinoma of the pancreas. Hepatogastroenterology, 55(85):1242-1245.

[20] Tjon, A.T.R.T., Jansen, J.B., Falke, T.H., Lamers, C.B., 1994. Imaging features of somatostatinoma: MR, CT, US, and angiography. J. Comput. Assist. Tomogr., 18(3):427-431.

[21] Tomono, H., Kitamura, H., Iwase, M., Kuze, S., Toyoda, F., Mori, N., Tamoto, E., Inuzuka, K., Fujita, H., Konishi, Y., Naito, M., Tanioka, F., 2003. A small, incidentally detected pancreatic somatostatinoma: report of a case. Surg. Today, 33(1):62-65.

[22] Vinik, A.I., Strodel, W.E., Eckhauser, F.E., Moattari, A.R., Lloyd, R., 1987. Somatostatinomas, ppomas, neurotensinomas. Semin. Oncol., 14(3):263-281.

[23] Zhang, Z.Y., Zhang, R., Wang, L., Shen, C., Yan, J.Q., Chen, Y.J., Li, Q.Y., Yang, W.P., Peng, C.H., Li, H.W., Zhou, G.W., 2008. Diagnosis and treatment of pancreatic somatostatinoma: a case report. Chin. Med. J. (Engl.), 121(22):2363-2365.