Abstract
Background
Somatostatinomas involving the gastrointestinal tract are extremely rare neoplasms that typically present with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize the somatostatinoma syndrome. Because of a relatively large size at the time of presentation (average diameter of 5 cm) and common location within the head of the pancreas, the Whipple procedure (pancreaticoduodenectomy) serves as the predominant modality for curative and palliative surgical approaches.
Methods
Two cases of somatostatinoma involving the minor duodenal papilla with concomitant pancreatic divisum were reviewed, with a general overview of this unique islet cell tumor.
Results
Unlike typical somatostatinomas, these two tumors were subcentimeter in size but were associated with synchronous regional metastasis.
Conclusions
Somatostatinomas are often associated with regional and/or portal metastases at the time of diagnosis, and only 60% to 70% of surgical cases result in complete tumor resection. Predictors of an unfavorable prognosis include size >3 cm, poor cytological differentiation, regional and/or portal metastasis, and incomplete surgical resection. Even in the presence of synchronous metastases, the 5-year overall survival for patients with somatostatinoma is approximately 40%. Currently, there are no clinical trials demonstrating significant improvement in survival with the use of adjuvant therapy.
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References
Pearse AG, The APUD concept and its implications in pathology.Pathol Annu 1974;9:27–41.
Konomi K, Chijiiwa K, Katsuta T, Yamaguchi K. Pancreatic somatostatinoma: a case report and review of the literature.J Surg Oncol 1990;43:259–65.
Wynick D, Williams SJ, Bloom SR. Symptomatic secondary hormone syndromes in patients with established malignant pancreatic endocrine tumors.N Engl J Med 1988;319:605–7.
Anene C, Thompson JS, Saigh J, Badakhsh S, Ecklund RE. Somatostationoma: atypical presentation of a rare pancreatic tumor.Am J Gastroenterol 1995;90:819–21.
Harris GJ, Tio F, Cruz AB. Somatostatinoma: a case report and review of the literature.J Surg Oncol 1987;36:8–16.
Larsson LI, Holst JJ, Kuhl C, et al. Pancreatic somatostationoma: clinical features and physiological implications.Lancet 1977;1:666–8.
Ganda OP, Weir GC, Soeldner JS, et al. Somatostatinoma. A somatostatin-containing tumor of the endocrine pancreas.N Engl J Med 1979;296:963–7.
Krejs GJ, Orcil L, Conlon JM. Somatostatinoma syndrome: biochemical, morphologic, and chemical features.N Engl J Med 1979;301:285–92.
Delcore R, Friesen SR. Gastrointestinal neuroendocrine tumors.J Am Coll Surg 1994;178:187–211.
Malone MJ, Silverman ML, Braasch JW, Jin G, Dayal Y. Early somatostatinoma of the duct of Santorini.Arch Surg 1985;120:1381–3.
Vinik AI, Moattari AR. Treatment of endocrine tumors of the pancreas.Endocrinol Metab Clin North Am 1989;18:483–518.
Griffiths DFR, Williams GT, Williams ED. Duodenal carcinoid tumours, phaeochromocytoma and neurofibromatosis: islet cell tumour, phaeochromocytoma and the von Hippel-Lindau complex—two distinctive neuroendocrine syndromes.Q J Med 1987; 64:769–82.
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;18:13–22.
Lucey R, Yamada T. Biochemistry and physiology of gastrointestinal somatostatin.Dig Dis Sci 1989;34:55–135.
Dayal Y, Ganda OP.Somatostatin-Producing Tumors. Endocrine Pathology of the Gut and Pancreas. Boca Raton, FL: CRC Press, 1991:241–77.
Reubi JC. Neuropeptide receptors in health and disease: the molecular basis for in vovo imaging.J Nucl Med 1995;36:1825–35.
Angeletti S, Corleto VD, Schillaci O, et al. Use of the somatostatin analogue octreotide to localise and manage somatostatin-producing tumours.Gut 1998;42:792–4.
Estes BL, Krejs GJ. The inhibitory syndrome: somatostatinoma. In: Friesen SR, Thompson NW, eds.Surgical Endocrinology: Clinical Syndromes. Philadelphia: Lippincott, 1990:249–66.
Guo M, Lemos LB, Bigler S, Baliga M. Duodenal somatostatinoma of the ampulla of Vater diagnosed by endoscopic fine needle aspiration: a case report.Acta Cytol 2001;45:622–6.
Kent RB, van Heerden JA, Weiland LH. Non-functioning islet cell tumors.Ann Surg 1980;22:185–89.
Madeira I, Terris B, Voss M, et al. Prognostic factors with endocrine tumours of the duodenopancreatic area.Gut 1998;43:422–7.
Chamberlain RS, Canes D, Brown KT. Hepatic metastasis: does intervention alter outcome?J Am Coll Surg 2000;190:432–5.
Doussett B, Saint-Marre O, Pitre J. Metastatic endocrine tumors: medical treatment, surgical resections, or liver transplantation.World J Surg 1996;20:908–15.
Norton JA, Sugarbaker PH, Doppman JL. Aggressive resection of metastatic disease in selected patients with malignant gastrinoma.Ann Surg 1986;293:353–9.
Eriksson B, Oberg K, Skogseid B. Neuroendocrine pancreatic tumors, clinical findings in a prospective study of 84 patients.Acta Oncol 1989;3:373–7.
Weber HC, Venzon DJ, Lin JT. Deteminants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study.Gastroenterology 1995;108:1637–49.
Tanaka S, Yamashaki S, Matsushita H. Duodenal somatostatinoma: a case report and review of 31 cases with special referende to the relationship between tumor size and metastasis.Pathol Int 2000;50:146–52.
Moertel CG, Lefkopoulo M, Lipsitz S. Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma.N Engl J Med 1992;326:519–23.
Bukowski RM, Tangen CM, Peterson RF. Phase II trial of DTIC in patients with metastic carcinoid: a Southwest Oncology Group study.Cancer 1994;73:1505–8.
Eriksson B, Oberg K. An update of the medical treatment of malignant endocrine pancreatic tumors.Acta Oncol 1993;32:203–8.
Brentjens R, Saltz L. Islet cell tumors of the pancreas: the medical oncologist's perspective.Surg Clin North Am 2001;81:527–42.
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House, M.G., Yeo, C.J. & Schulick, R.D. Periampullary pancreatic somatostatinoma. Annals of Surgical Oncology 9, 869–874 (2002). https://doi.org/10.1007/BF02557523
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DOI: https://doi.org/10.1007/BF02557523