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Somatostatinoma

  • James M. L. WilliamsonEmail author

Abstract

Somatostatinomas are rare neuroendocrine tumours (NETs) that arise in the pancreas or duodenum. They are the fifth most common pancreatoduodenal NET (after insulinomas, non-functioning tumours, gastrinomas and VIPomas), with an incidence of 1 in 40 million [1–3]. They tend to be non-functioning, although they are rarely active and can produce a spectrum of symptoms called ‘somatostatinoma syndrome’ [4–6]. A degree of discord exists regarding the functional component of a somatostatinoma; some clinicians argue that a ‘syndrome’ of clinical symptoms must be present for the diagnosis of somatostatinoma and others that the diagnosis is purely immunohistochemical [7]. As most of these tumours are biochemically inert, they are either detected incidentally or by causing mass-effect pressure symptoms, and most will have metastasised by the time of diagnosis [6, 8, 9].

Keywords

Hepatic Metastasis Peptide Receptor Radionuclide Therapy Operative Resection Hepatic Artery Embolisation Rare Neuroendocrine Tumour 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Ganda OP, Weir GC, Soeldner JS et al (1977) “Somatostatinoma”: a somatostatin-containing tumor of the endocrine pancreas. N Engl J Med 296:963–967CrossRefPubMedGoogle Scholar
  2. 2.
    House MG, Yeo CJ, Schulick RD (2002) Periampullary pancreatic somatostatinoma. Ann Surg Oncol 9:869–874CrossRefPubMedGoogle Scholar
  3. 3.
    Williamson JML, Thorn CC, Spalding D, Williamson RCN (2011) Pancreatic and peripancreatic somatostatinomas. Ann R Coll Surg Engl 93:356–360CrossRefPubMedCentralPubMedGoogle Scholar
  4. 4.
    Ellison TA, Edil BH (2012) The current management of pancreatic neuroendocrine tumours. Adv Surg 46:283–296CrossRefPubMedGoogle Scholar
  5. 5.
    O’Toole D, Salazar R, Falconi M et al, Frascati Consensus Conference; European Neuroendocrine Tumor Society (2006) Rare functioning pancreatic endocrine tumors. Neuroendocrinology 84:189–195Google Scholar
  6. 6.
    Vinik AI, Woltering EA, Warner RRP et al (2010) NANETS Guidelines: NANETS consensus guidelines for the diagnosis of neuroendocrine tumor. Pancreas 39:713–734CrossRefPubMedGoogle Scholar
  7. 7.
    Metz DE, Jensen RT (2008) Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology 135:1469–1492CrossRefPubMedCentralPubMedGoogle Scholar
  8. 8.
    Vinik AI, Strodel WE, Eckhauser FE, Moattari AR, Lloyd R (1987) Somatostatinomas, PPomas, neurotensinomas. Semin Oncol 14:263–281PubMedGoogle Scholar
  9. 9.
    Doherty GM (2005) Rare endocrine tumours of the GI tract. Best Pract Res Clin Gastroenterol 19:807–817CrossRefPubMedGoogle Scholar
  10. 10.
    Kulke MH, Anthony LB, Bushnell DL et al (2010) NANETS treatment guidelines: well-differentiated neuroendocrine tumors of the stomach and pancreas. Pancreas 39:735–752CrossRefPubMedCentralPubMedGoogle Scholar
  11. 11.
    Steinmuller T, Kianmanesh R, Falconi M et al (2008) Consensus guidelines for the management of patients with liver metastases from digestive (neuro)endocrine tumours: foregut, midgut, hindgut and unknown primary. Neuroendocrinology 87:47–62CrossRefPubMedGoogle Scholar
  12. 12.
    Capurso G, Bettini R, Rinzivillo M et al (2011) Role of resection of the primary pancreatic neuroendocrine tumour only in patients with unresectable metastatic liver disease: a systemic review. Neuroendocrinology 93:223–229CrossRefPubMedGoogle Scholar
  13. 13.
    Klöppel G (2007) Tumour biology and histopathology of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 21:15–31CrossRefPubMedGoogle Scholar
  14. 14.
    Mao C, Shah A, Hanson DJ, Howard JM (1995) Von Recklinghausen’s disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas. J Surg Oncol 59:67–73CrossRefPubMedGoogle Scholar
  15. 15.
    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 18:13–22PubMedGoogle Scholar
  16. 16.
    Ectors N (1999) Pancreatic endocrine tumors: diagnostic pitfalls. Hepatogastroenterology 46:679–690PubMedGoogle Scholar
  17. 17.
    Harris GJ, Tio F, Cruz AB Jr (1987) Somatostatinoma: a case report and review of the literature. J Surg Oncol 36:8–16CrossRefPubMedGoogle Scholar
  18. 18.
    Moayedoddin B, Booya F, Wermers RA, Lloyd RV, Rubin J, Thompson GB, Fatourechi V (2006) Spectrum of malignant somatostatin-producing neuroendocrine tumors. Endocr Pract 12:394–400CrossRefPubMedGoogle Scholar
  19. 19.
    Garbrecht N, Anlauf M, Schmitt A et al (2008) Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behaviour, association with inherited syndromes, and functional activity. Endocr Relat Cancer 15:229–241CrossRefPubMedGoogle Scholar
  20. 20.
    Oberg K, Eriksson B (2005) Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol 19:753–781CrossRefPubMedGoogle Scholar
  21. 21.
    Cappelli C, Agosti B, Braga M, Cumetti D, Gandossi E, Rizzoni D, Agabiti RE (2004) Von Recklinghausen’s neurofibromatosis associated with duodenal somatostatinoma. A case report and review of the literature. Minerva Endocrinol 29:19–24PubMedGoogle Scholar
  22. 22.
    Karasawa Y, Sakaguchi M, Minami S, Kitano K, Kawa S, Aoki Y, Itoh N, Sakurai A, Miyazaki M, Watanabe T, Akimoto M, Arakura N, Kiyosawa K (2001) Duodenal somatostatinoma and erythrocytosis in a patient with von Hippel-Lindau disease type 2A. Intern Med 40:38–43CrossRefPubMedGoogle Scholar
  23. 23.
    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 34:661–665CrossRefPubMedGoogle Scholar
  24. 24.
    Sreenarasimhaiah J, Armstrong LA, Tang SJ, Barnett C (2009) Pancreatic somatostatinoma and tuberous sclerosis: an exceedingly rare association. Gastrointest Endosc 69:379–381CrossRefPubMedGoogle Scholar
  25. 25.
    Barahona-Garrido J, Aguirre-Gutiérrez R, Gutiérrez-Manjarrez JI, Tellez-Avila FI, López-Arce G, Fomperoza-Torres A, Criales S, Sánchez-Cortés E, Sarti HM, Yamamoto-Furusho JK (2009) Association of GIST and somatostatinoma in a patient with type-1 neurofibromatosis: is there a common pathway? Am J Gastroenterol 104:797–799CrossRefPubMedGoogle Scholar
  26. 26.
    Sakorafas GH, Giannopoulos GA, Parasi A, Konstantoudakis G, Tzanakis N, Stergiopoulos S, Peros G (2008) Large somatostatin-producing endocrine carcinoma of the ampulla of vater in association with GIST in a patient with von Recklinghausen’s disease. Case report and review of the literature. JOP 9:633–639PubMedGoogle Scholar
  27. 27.
    de Herder WW, Hofland LJ, van der Lely AJ, Lamberts SWJ (2003) Somatostatin receptors in gastroenteropancreatic neuroendocrine tumours. Endocr Relat Cancer 10:451–458CrossRefPubMedGoogle Scholar
  28. 28.
    Yamada Y, Stoffel M, Espinosa R 3rd, Xiang KS, Seino M, Seino S, Le Beau MM, Bell GI (1993) Human somatostatin receptor genes: localization to human chromosomes 14, 17, and 22 and identification of simple tandem repeat polymorphisms. Genomics 15:449–452CrossRefPubMedGoogle Scholar
  29. 29.
    Volante M, Bozzalla-Cassione F, Papotti M (2004) Somatostatin receptors and their interest in diagnostic pathology. Endocr Pathol 15:275–292CrossRefPubMedGoogle Scholar
  30. 30.
    Corleto VD, Falconi M, Panzuto F et al (2009) Somatostatin receptor subtypes 2 and 5 are associated with better survival in well-differentiated endocrine carcinomas. Neuroendocrinology 82:223–230CrossRefGoogle Scholar
  31. 31.
    Gibril F, Jensen R (2004) Diagnostic uses of radiolabelled somatostatin-receptor analogues in gastroenteropancreatic endocrine tumors. Dig Liver Dis 36:S106–S120CrossRefPubMedGoogle Scholar
  32. 32.
    Capurso G, Panzuto F, Delle Fave G (2010) Clinical relevance of the expression of somatostatin receptors in digestive endocrine tumours. Dig Liver Dis 42:173–174CrossRefPubMedGoogle Scholar
  33. 33.
    Kam BLR, Teunissen JJM, Krenning EP, de Herder WW, Khan S, van Vilet EI, Kwekkeboom DJ (2012) Lutetium-labelled peptides for therapy of neuroendocrine tumours. Eur J Nucl Med Mol Imaging 39:S103–S112CrossRefPubMedGoogle Scholar
  34. 34.
    Klimstra DS, Modlin IR, Adsay NV et al (2010) Pathological reporting of neuroendocrine tumors: application of the Delphic consensus process to the development of a minimum pathology data set. Am J Surg Pathol 34:300–313CrossRefPubMedGoogle Scholar
  35. 35.
    NANETS Guidelines (2010) The pathological classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas 39:707–712CrossRefGoogle Scholar
  36. 36.
    Rindi G, Klöppel G, Alhman H et al, all other Frascati Consensus Conference participants; European Neuroendocrine Tumor Society (ENETS) (2006) TNM staging of foregut (neuro)endocrine tumours: a consensus proposal including a grading system. Virchows Arch 449:395–401Google Scholar
  37. 37.
    Sorbin LH, Wittekind C (eds) (2002) TNM classification of malignant tumours. Wiley-Liss, New YorkGoogle Scholar
  38. 38.
    King CM, Reznek RH, Dacie JE, Wass JA (1994) Imaging islet cell tumours. Clin Radiol 49:295–303CrossRefPubMedGoogle Scholar
  39. 39.
    Rockall AG, Reznek RH (2007) Imaging of neuroendocrine tumours (CT/MR/US). Best Pract Res Clin Endocrinol Metab 21:43–68CrossRefPubMedGoogle Scholar
  40. 40.
    Debray MP, Geoffroy O, Laissy JP, Lebtahi R, Sibermann-Hoffman O, Henry-Feugeas MC, Cadiot G, Mignon M, Schouman-Claeys E (2001) Imaging appearances of metastases from neuroendocrine tumours of the pancreas. Br J Radiol 74:1065–1070CrossRefPubMedGoogle Scholar
  41. 41.
    Angeletti S, Corleto VD, Schillaci O, Marignani M, Annibale B, Moretti A, Silecchia G, Scopinaro F, Basso N, Bordi C, Delle Fave G (1998) Use of the somatostatin analogue octreotide to localise and manage somatostatin-producing tumours. Gut 42:792–794CrossRefPubMedCentralPubMedGoogle Scholar
  42. 42.
    Reubi JC (1995) Neuropeptide receptors in health and disease: the molecular basis for in vivo imaging. J Nucl Med 36:825–835Google Scholar
  43. 43.
    Termanini B, Gibril F, Reynolds JC, Doppman JL, Chen CC, Stewart CA, Sutliff VE, Jensen RT (1997) Value of somatostatin receptor scintigraphy: a prospective study in gastrinoma of its effect on clinical management. Gastroenterology 112:335–347CrossRefPubMedGoogle Scholar
  44. 44.
    Krenning EP, Kwekkeboom DJ, Oei HY, de Jong RJ, Dop FJ, Reubi JC, Lamberts SW (1994) Somatostatin-receptor scintigraphy in gastroenteropancreatic tumors. An overview of European results. Ann N Y Acad Sci 733:416–424CrossRefPubMedGoogle Scholar
  45. 45.
    O’Grady HL, Conlon KC (2008) Pancreatic neuroendocrine tumours. Eur J Surg Oncol 34:424–432Google Scholar
  46. 46.
    McClean AM, Fairclough PD (2005) Endoscopic ultrasound in the localisation of pancreatic islet cell tumours. Best Pract Res Clin Endocrinol Metab 19:177–193CrossRefGoogle Scholar
  47. 47.
    O’Brien TD, Chejfec G, Prinz RA (1993) Clinical features of duodenal somatostatinomas. Surgery 114:1144–1147PubMedGoogle Scholar
  48. 48.
    Saif MW (2011) Pancreatic neoplasm in 2011: an update. JOP 12:316–321PubMedGoogle Scholar
  49. 49.
    Kwekkeboom DJ, de Herder WW, Kam BL et al (2008) Treatment with the radiolabelled somatostatin analog [177Lu-DOTA0, Tyr3]octreotate: toxicity, efficacy and survival. J Clin Oncol 26:2124–2130CrossRefPubMedGoogle Scholar
  50. 50.
    Chamberlain RS, Canes D, Brown KT, Saltz L, Jarnagin W, Fong Y, Blumgart LH (2000) Hepatic neuroendocrine metastases: does intervention alter outcome? J Am Coll Surg 4:432–445CrossRefGoogle Scholar
  51. 51.
    Norton JA (2005) Endocrine tumours of the gastrointestinal tract. Surgical treatment of neuroendocrine metastases. Best Pract Clin Res Gastroenterol 19:577–583CrossRefGoogle Scholar
  52. 52.
    Que FG, Sarmiento JM, Nogorney DM (2006) Hepatic surgery for metastatic gastrointestinal tumours. Adv Exp Med Biol 574:43–56PubMedGoogle Scholar
  53. 53.
    Chamberlain RS, Canes D, Brown KT, Saltz L, Jarnagin W, Fong Y, Blumgart LH (2000) Hepatic neuroendocrine metastases: does intervention alter outcomes? J Am Coll Surg 190:432–445CrossRefPubMedGoogle Scholar
  54. 54.
    Moretel CG, Lefkopoulo M, Lipsitz S, Hagn RG, Klaassen D (1992) Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 326:519–523CrossRefGoogle Scholar
  55. 55.
    Chen H, Hardacre JM, Uzar A, Cameron JL, Choti MA (1998) Isolated liver metastases from neuroendocrine tumors: does resection prolong survival? J Am Coll Surg 187:88–92; discussion 92–93CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Hepato-pancreato-biliary surgeryBristol Royal InfirmaryBristolUK

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