Pancreatic Neuroendocrine Tumors

  • Thiruvengadam MunirajEmail author
  • Harry R. Aslanian
Living reference work entry


Neuroendocrine tumors (NETs) are a relatively rare and heterogeneous group, comprising about 2% of all malignancies with almost 2/3 of NETs being nonfunctional tumors. The incidence increases with age. Most non-appendix, non-pancreas NETs are seen in age over 60 years. Diagnosis of NET is often challenging and requires careful clinical history along with laboratory tests and an array of imaging procedures. While functioning NETs could present with symptoms related to hormone hypersecretion, non-functioning NETs often present incidentally or due to mass effect. Surgical resection remains the treatment of choice even with metastatic disease. The newer diagnostic and treatment modalities offer potential treatment and prolong disease-free survival for patients with improvement in quality of life.


Pancreatic Neuroendocrine Cancer Staging Confocal Endoscopic ultrasound Octreoscan MEN Neuroendocrine tumors and management 


  1. Akerstrom G, Hellman P. Surgery on neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007;21:87–109.PubMedCrossRefGoogle Scholar
  2. American Cancer Society. Pancreatic cancer survival by stage. 2012
  3. Basu B, Sirohi B, Corrie P. Systemic therapy for neuroendocrine tumours of gastroenteropancreatic origin. Endocr Relat Cancer. 2010;17:R75–90.PubMedCrossRefGoogle Scholar
  4. Bosman FT, Carneiro F, Hruban RH, Theise ND. Tumours of the pancreas. Lyon: IARC Press; 2010.Google Scholar
  5. Boukhman MP, et al. Localization of insulinomas. Arch Surg. 1999;134:818–22; discussion 822–813.PubMedCrossRefGoogle Scholar
  6. Boyce M, Thomsen L. Gastric neuroendocrine tumors: prevalence in Europe, USA, and Japan, and rationale for treatment with a gastrin/CCK2 receptor antagonist. Scand J Gastroenterol. 2015;50:550–9.PubMedCrossRefGoogle Scholar
  7. Brizi MG, et al. High resolution spiral computed tomography of the pancreas. Rays. 2001;26:111–5.PubMedGoogle Scholar
  8. Cadiot G, et al. Preoperative detection of duodenal gastrinomas and peripancreatic lymph nodes by somatostatin receptor scintigraphy. Groupe D’etude Du Syndrome De Zollinger-Ellison. Gastroenterology. 1996;111:845–54.PubMedCrossRefGoogle Scholar
  9. Campana D, et al. Chromogranin A: is it a useful marker of neuroendocrine tumors? J Clin Oncol. 2007;25:1967–73.PubMedCrossRefGoogle Scholar
  10. Chamberlain RS, et al. Hepatic neuroendocrine metastases: does intervention alter outcomes? J Am Coll Surg. 2000;190:432–45.PubMedCrossRefGoogle Scholar
  11. Chatzipantelis P, et al. Endoscopic ultrasound-guided fine-needle aspiration cytology of pancreatic neuroendocrine tumors: a study of 48 cases. Cancer. 2008;114:255–62.PubMedCrossRefGoogle Scholar
  12. Corleto VD, Delle Fave G, Jensen RT. Molecular insights into gastrointestinal neuroendocrine tumours: importance and recent advances. Dig Liver Dis. 2002;34:668–80.PubMedCrossRefGoogle Scholar
  13. Dabizzi E, Panossian A, Raimondo M. Management of pancreatic neuroendocrine tumors. Minerva Gastroenterol Dietol. 2010;56:467–79.PubMedGoogle Scholar
  14. de Herder WW. Biochemistry of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007;21:33–41.PubMedCrossRefGoogle Scholar
  15. Doppman JL, et al. Insulinomas: localization with selective intraarterial injection of calcium. Radiology. 1991;178:237–41.PubMedCrossRefGoogle Scholar
  16. Doppman JL, et al. Localization of insulinomas to regions of the pancreas by intra-arterial stimulation with calcium. Ann Intern Med. 1995;123:269–73.PubMedCrossRefGoogle Scholar
  17. Dromain C, et al. Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging. J Clin Oncol. 2005;23:70–8.PubMedCrossRefGoogle Scholar
  18. Duerr EM, Chung DC. Molecular genetics of neuroendocrine tumors. Best Pract Res Clin Endocrinol Metab. 2007;21:1–14.PubMedCrossRefGoogle Scholar
  19. Duran I, et al. A phase II clinical and pharmacodynamic study of temsirolimus in advanced neuroendocrine carcinomas. Br J Cancer. 2006;95:1148–54.PubMedPubMedCentralCrossRefGoogle Scholar
  20. Edge SB, Compton CC. The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM. Ann Surg Oncol. 2010;17:1471–4.CrossRefGoogle Scholar
  21. Falconi M, et al. Well-differentiated pancreatic nonfunctioning tumors/carcinoma. Neuroendocrinology. 2006;84:196–211.PubMedCrossRefGoogle Scholar
  22. Fendrich V, Waldmann J, Bartsch DK, Langer P. Surgical management of pancreatic endocrine tumors. Nat Rev Clin Oncol. 2009;6:419–28.PubMedCrossRefGoogle Scholar
  23. Fidler JL, Johnson CD. Imaging of neuroendocrine tumors of the pancreas. Int J Gastrointest Cancer. 2001;30:73–85.PubMedCrossRefGoogle Scholar
  24. Fink G, et al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest. 2001;119:1647–51.PubMedCrossRefGoogle Scholar
  25. Forrer F, Valkema R, Kwekkeboom DJ, de Jong M, Krenning EP. Neuroendocrine tumors. Peptide receptor radionuclide therapy. Best Pract Res Clin Endocrinol Metab. 2007;21:111–29.PubMedCrossRefGoogle Scholar
  26. Fottner C, et al. In vivo molecular imaging of somatostatin receptors in pancreatic islet cells and neuroendocrine tumors by miniaturized confocal laser-scanning fluorescence microscopy. Endocrinology. 2010;151:2179–88.PubMedCrossRefGoogle Scholar
  27. Frucht H, et al. Detection of duodenal gastrinomas by operative endoscopic transillumination. A prospective study. Gastroenterology. 1990;99:1622–7.PubMedCrossRefGoogle Scholar
  28. Gibril F, Jensen RT. Zollinger-Ellison syndrome revisited: diagnosis, biologic markers, associated inherited disorders, and acid hypersecretion. Curr Gastroenterol Rep. 2004;6:454–63.PubMedCrossRefGoogle Scholar
  29. Gibril F, Venzon DJ, Ojeaburu JV, Bashir S, Jensen RT. Prospective study of the natural history of gastrinoma in patients with MEN1: definition of an aggressive and a nonaggressive form. J Clin Endocrinol Metab. 2001;86:5282–93.PubMedCrossRefGoogle Scholar
  30. Gill GV, Rauf O, MacFarlane IA. Diazoxide treatment for insulinoma: a national UK survey. Postgrad Med J. 1997;73:640–1.PubMedPubMedCentralCrossRefGoogle Scholar
  31. Goetz M, Kiesslich R. Advances of endomicroscopy for gastrointestinal physiology and diseases. Am J Physiol Gastrointest Liver Physiol. 2010;298:G797–806.PubMedCrossRefPubMedCentralGoogle Scholar
  32. Goudet P, et al. Risk factors and causes of death in MEN1 disease. A GTE (Groupe d’Etude des Tumeurs Endocrines) cohort study among 758 patients. World J Surg. 2010;34:249–55.PubMedCrossRefPubMedCentralGoogle Scholar
  33. Grant CS. Surgical management of malignant islet cell tumors. World J Surg. 1993;17:498–503.PubMedCrossRefPubMedCentralGoogle Scholar
  34. Grant CS. Insulinoma. Best Pract Res Clin Gastroenterol. 2005;19:783–98.PubMedCrossRefPubMedCentralGoogle Scholar
  35. Gray’s anatomy: the anatomical basis of clinical practice. (ed. DSc, S.S.P.) 1562 (Elsevier, 2015).Google Scholar
  36. Gregoire E, Le Treut YP. Liver transplantation for primary or secondary endocrine tumors. Transpl Int. 2010;23:704–11.PubMedCrossRefPubMedCentralGoogle Scholar
  37. Halperin DM, Kulke MH. Management of pancreatic neuroendocrine tumors. Gastroenterol Clin N Am. 2012;41:119–31.CrossRefGoogle Scholar
  38. Hellman P, et al. Surgical strategy for large or malignant endocrine pancreatic tumors. World J Surg. 2000;24:1353–60.PubMedCrossRefPubMedCentralGoogle Scholar
  39. Hellman P, Hennings J, Akerstrom G, Skogseid B. Endoscopic ultrasonography for evaluation of pancreatic tumours in multiple endocrine neoplasia type 1. Br J Surg. 2005;92:1508–12.PubMedCrossRefPubMedCentralGoogle Scholar
  40. Hill JS, et al. Pancreatic neuroendocrine tumors: the impact of surgical resection on survival. Cancer. 2009;115:741–51.PubMedCrossRefPubMedCentralGoogle Scholar
  41. Hirshberg B, et al. Blind distal pancreatectomy for occult insulinoma, an inadvisable procedure. J Am Coll Surg. 2002;194:761–4.PubMedCrossRefPubMedCentralGoogle Scholar
  42. Hobday TJ, Rubin J, Holen K, Picus J, Donehower R, Marschke R, Maples W, Lloyd R, Mahoney M, Erlichman C. MC044h, a phase II trial of sorafenib in patients (pts) with metastatic neuroendocrine tumors (NET): a Phase II Consortium (P2C) study. In: 2007 ASCO annual meeting proceedings, vol. 25, p. 4504. J Clin Oncol, 2007.CrossRefGoogle Scholar
  43. Huai JC, et al. Localization and surgical treatment of pancreatic insulinomas guided by intraoperative ultrasound. Am J Surg. 1998;175:18–21.PubMedCrossRefPubMedCentralGoogle Scholar
  44. Imamura M, et al. New pancreas-preserving total duodenectomy technique. World J Surg. 2005;29:203–7.PubMedCrossRefPubMedCentralGoogle Scholar
  45. Imamura M, Komoto I, Ota S. Changing treatment strategy for gastrinoma in patients with Zollinger-Ellison syndrome. World J Surg. 2006;30:1–11.PubMedCrossRefPubMedCentralGoogle Scholar
  46. Ito H, et al. Surgery and staging of pancreatic neuroendocrine tumors: a 14-year experience. J Gastrointest Surg. 2010;14:891–8.PubMedCrossRefPubMedCentralGoogle Scholar
  47. Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J. Harrison’s principles of internal medicine. United States of America: The McGraw-Hill Companies; 2012.Google Scholar
  48. Jensen RT, et al. Gastrinoma (duodenal and pancreatic). Neuroendocrinology. 2006;84:173–82.PubMedCrossRefPubMedCentralGoogle Scholar
  49. Jensen RT, Berna MJ, Bingham DB, Norton JA. Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer. 2008;113:1807–43.PubMedPubMedCentralCrossRefGoogle Scholar
  50. Kaltsas GA, Besser GM, Grossman AB. The diagnosis and medical management of advanced neuroendocrine tumors. Endocr Rev. 2004;25:458–511.PubMedCrossRefPubMedCentralGoogle Scholar
  51. Kato M, et al. Curative resection of microgastrinomas based on the intraoperative secretin test. World J Surg. 2000;24:1425–30.PubMedCrossRefPubMedCentralGoogle Scholar
  52. King CM, Reznek RH, Dacie JE, Wass JA. Imaging islet cell tumours. Clin Radiol. 1994;49:295–303.PubMedCrossRefPubMedCentralGoogle Scholar
  53. Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol. 2007;20(Suppl 1):S94–112.PubMedCrossRefPubMedCentralGoogle Scholar
  54. Klimstra DS, et al. Pathology reporting of neuroendocrine tumors: application of the Delphic consensus process to the development of a minimum pathology data set. Am J Surg Pathol. 2010;34:300–13.PubMedCrossRefPubMedCentralGoogle Scholar
  55. Kloppel G, Perren A, Heitz PU. The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification. Ann N Y Acad Sci. 2004;1014:13–27.PubMedPubMedCentralCrossRefGoogle Scholar
  56. Konda VJ, et al. First assessment of needle-based confocal laser endomicroscopy during EUS-FNA procedures of the pancreas (with videos). Gastrointest Endosc. 2011;74:1049–60.PubMedCrossRefPubMedCentralGoogle Scholar
  57. Kouvaraki MA, et al. Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg. 2006;30:643–53.PubMedCrossRefPubMedCentralGoogle Scholar
  58. Krausz Y, et al. SPECT/CT hybrid imaging with 111In-pentetreotide in assessment of neuroendocrine tumours. Clin Endocrinol. 2003;59:565–73.CrossRefGoogle Scholar
  59. Krenning EP, et al. Somatostatin receptor scintigraphy with [111In-DTPA-D-Phe1]- and [123I-Tyr3]-octreotide: the Rotterdam experience with more than 1000 patients. Eur J Nucl Med. 1993;20:716–31.PubMedCrossRefPubMedCentralGoogle Scholar
  60. Kulke MH, et al. NANETS treatment guidelines: well-differentiated neuroendocrine tumors of the stomach and pancreas. Pancreas. 2010;39:735–52.PubMedPubMedCentralCrossRefGoogle Scholar
  61. Kulke MH, et al. Future directions in the treatment of neuroendocrine tumors: consensus report of the National Cancer Institute Neuroendocrine Tumor clinical trials planning meeting. J Clin Oncol. 2011;29:934–43.PubMedPubMedCentralCrossRefGoogle Scholar
  62. Kwekkeboom DJ, et al. Treatment with the radiolabeled somatostatin analog [177 Lu-DOTA 0,Tyr3]octreotate: toxicity, efficacy, and survival. J Clin Oncol. 2008;26:2124–30.PubMedCrossRefPubMedCentralGoogle Scholar
  63. Lamberts SW, Bakker WH, Reubi JC, Krenning EP. Somatostatin-receptor imaging in the localization of endocrine tumors. N Engl J Med. 1990;323:1246–9.PubMedCrossRefPubMedCentralGoogle Scholar
  64. Langer P, et al. Prospective evaluation of imaging procedures for the detection of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. World J Surg. 2004;28:1317–22.PubMedCrossRefPubMedCentralGoogle Scholar
  65. Lawrence B, et al. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin N Am. 2011;40:1–18. , vii.CrossRefGoogle Scholar
  66. Le Treut YP, et al. Predictors of long-term survival after liver transplantation for metastatic endocrine tumors: an 85-case French multicentric report. Am J Transplant. 2008;8:1205–13.PubMedCrossRefPubMedCentralGoogle Scholar
  67. Legmann P, et al. Pancreatic tumors: comparison of dual-phase helical CT and endoscopic sonography. AJR Am J Roentgenol. 1998;170:1315–22.PubMedCrossRefPubMedCentralGoogle Scholar
  68. Liszka L, et al. Discrepancies between two alternative staging systems (European Neuroendocrine Tumor Society 2006 and American Joint Committee on Cancer/Union for International Cancer Control 2010) of neuroendocrine neoplasms of the pancreas. A study of 50 cases. Pathol Res Pract. 2011;207:220–4.PubMedCrossRefPubMedCentralGoogle Scholar
  69. Mertz H, Gautam S. The learning curve for EUS-guided FNA of pancreatic cancer. Gastrointest Endosc. 2004;59:33–7.PubMedCrossRefPubMedCentralGoogle Scholar
  70. Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 2008;135:1469–92.PubMedPubMedCentralCrossRefGoogle Scholar
  71. Miederer M, Weber MM, Fottner C. Molecular imaging of gastroenteropancreatic neuroendocrine tumors. Gastroenterol Clin N Am. 2010;39:923–35.CrossRefGoogle Scholar
  72. Minter RM, Simeone DM. Contemporary management of nonfunctioning pancreatic neuroendocrine tumors. J Gastrointest Surg. 2012;16:435–46.PubMedCrossRefGoogle Scholar
  73. Modlin IM, Cornelius E, Lawton GP. Use of an isotopic somatostatin receptor probe to image gut endocrine tumors. Arch Surg. 1995;130:367–73; discussion 373–364.PubMedCrossRefGoogle Scholar
  74. Modlin IM, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9:61–72.PubMedCrossRefGoogle Scholar
  75. Moertel CG, Lefkopoulo M, Lipsitz S, Hahn RG, Klaassen D. Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med. 1992;326:519–23.PubMedCrossRefPubMedCentralGoogle Scholar
  76. Muniraj T, Vignesh S, Shetty S, Thiruvengadam S, Aslanian HR. Pancreatic neuroendocrine tumors. Dis Mon. 2013;59:5–19.PubMedCrossRefGoogle Scholar
  77. Norton JA. Surgery for primary pancreatic neuroendocrine tumors. J Gastrointest Surg. 2006;10:327–31.PubMedCrossRefPubMedCentralGoogle Scholar
  78. Norton JA, et al. Morbidity and mortality of aggressive resection in patients with advanced neuroendocrine tumors. Arch Surg. 2003;138:859–66.PubMedCrossRefPubMedCentralGoogle Scholar
  79. Norton JA, et al. Surgery increases survival in patients with gastrinoma. Ann Surg. 2006;244:410–9.PubMedPubMedCentralGoogle Scholar
  80. O’Grady HL, Conlon KC. Pancreatic neuroendocrine tumours. Eur J Surg Oncol. 2008;34:324–32.PubMedCrossRefGoogle Scholar
  81. Oronsky B, Ma PC, Morgensztern D, Carter CA. Nothing but NET: a review of neuroendocrine tumors and carcinomas. Neoplasia (New York, NY). 2017;19:991–1002.CrossRefGoogle Scholar
  82. Pape UF, et al. Prognostic relevance of a novel TNM classification system for upper gastroenteropancreatic neuroendocrine tumors. Cancer. 2008;113:256–65.PubMedCrossRefGoogle Scholar
  83. Phan AT, Yao JC, Fogelman DR, Hess KR, Ng CS, Bullock SA, Malinowski P, Regan E, Kulke M. A prospective, multi-institutional phase II study of GW786034 (pazopanib) and depot octreotide (sandostatin LAR) in advanced low-grade neuroendocrine carcinoma (LGNEC). In: ASCO annual meeting, vol. 28, p. 4001. J Clin Oncol 2010).CrossRefGoogle Scholar
  84. Plockinger U, et al. Guidelines for the diagnosis and treatment of neuroendocrine gastrointestinal tumours. A consensus statement on behalf of the European Neuroendocrine Tumour Society (ENETS). Neuroendocrinology. 2004;80:394–424.PubMedCrossRefGoogle Scholar
  85. Raymond E, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011;364:501–13.PubMedCrossRefGoogle Scholar
  86. Rindi G, et al. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2006;449:395–401.PubMedPubMedCentralCrossRefGoogle Scholar
  87. Rosch T, et al. Localization of pancreatic endocrine tumors by endoscopic ultrasonography. N Engl J Med. 1992;326:1721–6.PubMedCrossRefGoogle Scholar
  88. Roy PK, et al. Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore). 2000;79:379–411.CrossRefGoogle Scholar
  89. Saadettin Kilickap HKM. Neuroendocrine tumours diagnosis and management. New York: Springer; 2015.Google Scholar
  90. Sakurai A, et al. Long-term follow-up of patients with multiple endocrine neoplasia type 1. Endocr J. 2007;54:295–302.PubMedCrossRefGoogle Scholar
  91. Sarmiento JM, Que FG. Hepatic surgery for metastases from neuroendocrine tumors. Surg Oncol Clin N Am. 2003;12:231–42.PubMedCrossRefGoogle Scholar
  92. Scarpa A, et al. Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients. Mod Pathol. 2010;23:824–33.PubMedCrossRefGoogle Scholar
  93. Schillaci O, Corleto VD, Annibale B, Scopinaro F, Delle Fave G. Single photon emission computed tomography procedure improves accuracy of somatostatin receptor scintigraphy in gastro-entero pancreatic tumours. Ital J Gastroenterol Hepatol. 1999;31(Suppl 2):S186–9.PubMedGoogle Scholar
  94. Schott M, et al. Neuroendocrine neoplasms of the gastrointestinal tract. Dtsch Arztebl Int. 2011;108:305–12.PubMedPubMedCentralGoogle Scholar
  95. Schottelius M, et al. First (18)F-labeled tracer suitable for routine clinical imaging of sst receptor-expressing tumors using positron emission tomography. Clin Cancer Res. 2004;10:3593–606.PubMedCrossRefGoogle Scholar
  96. Strosberg J, Nasir A, Coppola D, Wick M, Kvols L. Correlation between grade and prognosis in metastatic gastroenteropancreatic neuroendocrine tumors. Hum Pathol. 2009;40:1262–8.PubMedCrossRefPubMedCentralGoogle Scholar
  97. Strosberg JR, et al. Prognostic validity of a novel American Joint Committee on Cancer Staging Classification for pancreatic neuroendocrine tumors. J Clin Oncol. 2011a;29:3044–9.PubMedCrossRefGoogle Scholar
  98. Strosberg JR, et al. First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer. 2011b;117:268–75.PubMedCrossRefGoogle Scholar
  99. Thom AK, et al. Prospective study of the use of intraarterial secretin injection and portal venous sampling to localize duodenal gastrinomas. Surgery. 1992;112:1002–8; discussion 1008–1009.PubMedGoogle Scholar
  100. Thomas-Marques L, et al. Prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. Am J Gastroenterol. 2006;101:266–73.PubMedCrossRefGoogle Scholar
  101. Thompson NW, Eckhauser FE. Malignant islet-cell tumors of the pancreas. World J Surg. 1984;8:940–51.PubMedCrossRefGoogle Scholar
  102. Thompson GB, van Heerden JA, Grant CS, Carney JA, Ilstrup DM. Islet cell carcinomas of the pancreas: a twenty-year experience. Surgery. 1988;104:1011–7.PubMedGoogle Scholar
  103. Toumpanakis C, Meyer T, Caplin ME. Cytotoxic treatment including embolization/chemoembolization for neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007;21:131–44.PubMedCrossRefGoogle Scholar
  104. Touzios JG, et al. Neuroendocrine hepatic metastases: does aggressive management improve survival? Ann Surg. 2005;241:776–83; discussion 783–775.PubMedPubMedCentralCrossRefGoogle Scholar
  105. Triponez F, et al. Is surgery beneficial for MEN1 patients with small (< or = 2 cm), nonfunctioning pancreaticoduodenal endocrine tumor? An analysis of 65 patients from the GTE. World J Surg. 2006;30:654–62; discussion 663–654.PubMedCrossRefGoogle Scholar
  106. Vagefi PA, et al. Evolving patterns in the detection and outcomes of pancreatic neuroendocrine neoplasms: the Massachusetts General Hospital experience from 1977 to 2005. Arch Surg. 2007;142:347–54.PubMedPubMedCentralCrossRefGoogle Scholar
  107. Van Hoe L, Gryspeerdt S, Marchal G, Baert AL, Mertens L. Helical CT for the preoperative localization of islet cell tumors of the pancreas: value of arterial and parenchymal phase images. AJR Am J Roentgenol. 1995;165:1437–9.PubMedCrossRefGoogle Scholar
  108. Wamsteker EJ, Gauger PG, Thompson NW, Scheiman JM. EUS detection of pancreatic endocrine tumors in asymptomatic patients with type 1 multiple endocrine neoplasia. Gastrointest Endosc. 2003;58:531–5.PubMedCrossRefGoogle Scholar
  109. Wang SC, et al. Identification of unknown primary tumors in patients with neuroendocrine liver metastases. Arch Surg. 2010;145:276–80.PubMedCrossRefGoogle Scholar
  110. Wolf P, et al. Clinical presentation in insulinoma predicts histopathological tumour characteristics. Clin Endocrinol. 2015;83:67–71.CrossRefGoogle Scholar
  111. Yang RH, Chu YK. Zollinger-Ellison syndrome: revelation of the gastrinoma triangle. Radiol Case Rep. 2015;10:827.PubMedPubMedCentralCrossRefGoogle Scholar
  112. Yao JC, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol. 2007;14:3492–500.PubMedPubMedCentralCrossRefGoogle Scholar
  113. Yao JC, et al. Daily oral everolimus activity in patients with metastatic pancreatic neuroendocrine tumors after failure of cytotoxic chemotherapy: a phase II trial. J Clin Oncol. 2010;28:69–76.PubMedCrossRefGoogle Scholar
  114. Yao JC, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011;364:514–23.PubMedPubMedCentralCrossRefGoogle Scholar
  115. Zatelli MC, et al. Chromogranin A as a marker of neuroendocrine neoplasia: an Italian Multicenter Study. Endocr Relat Cancer. 2007;14:473–82.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Yale University School of MedicineNew HavenUSA

Personalised recommendations