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Islets of Langerhans, 2. ed. pp 1–29Cite as

Pancreatic Neuroendocrine Tumors

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Abstract

Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms, with a prevalence of 1–2 per 100,000 people. Based on the presence or absence of a specific hormone-related clinical syndrome, they are divided into functioning and nonfunctioning. Among the former tumors, insulinomas are the most common. pNETs may be sporadic or associated with hereditary syndromes. The histopathology evaluation should include immunostaining with general (chromogranin A and synaptophysin) and specific neuroendocrine markers, as well as with the proliferation index Ki67. Chromogranin A is currently the most useful neuroendocrine biomarker for diagnosis and follow-up of pNETs. Other specific hormones released by the neoplastic cells can also be included in the biochemical evaluation. For tumor localization both noninvasive and invasive techniques may be used. Debulking procedures and medical therapy are the possible treatment options for pNETs, but surgery is the only modality that offers the possibility of cure.

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References

  • Ahlman H, Wangberg B et al (2000) Interventional treatment of gastrointestinal neuroendocrine tumours. Digestion 62(Suppl 1):59–68

    PubMed  Google Scholar 

  • Alexandraki KI, Kaltsas G (2012) Gastroenteropancreatic neuroendocrine tumors: new insights in the diagnosis and therapy. Endocrine 41(1):40–52

    PubMed  CAS  Google Scholar 

  • Al-Taan OS, Stephenson JA et al (2010) Laparoscopic pancreatic surgery: a review of present results and future prospects. HPB (Oxford) 12(4):239–243

    Google Scholar 

  • Anlauf M, Garbrecht N et al (2006) Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features. World J Gastroenterol 12(34):5440–5446

    PubMed  Google Scholar 

  • Arnold R (2007) Diagnosis and differential diagnosis of hypergastrinemia. Wien Klin Wochenschr 119(19–20):564–569

    PubMed  CAS  Google Scholar 

  • Banasch M, Schmitz F (2007) Diagnosis and treatment of gastrinoma in the era of proton pump inhibitors. Wien Klin Wochenschr 119(19–20):573–578

    PubMed  CAS  Google Scholar 

  • Biermasz NR, Smit JW et al (2007) Acromegaly caused by growth hormone-releasing hormone-producing tumors: long-term observational studies in three patients. Pituitary 10(3):237–249

    PubMed Central  PubMed  Google Scholar 

  • Bosman F, Carneiro F et al (eds) (2010) WHO classification of tumours of the digestive system. IARC Press, Lyon

    Google Scholar 

  • Boudreaux JP (2011) Surgery for gastroenteropancreatic neuroendocrine tumors (GEPNETS). Endocrinol Metab Clin North Am 40(1):163–171, ix

    PubMed  Google Scholar 

  • Buchmann I, Henze M et al (2007) Comparison of 68Ga-DOTATOC PET and 111In-DTPAOC (Octreoscan) SPECT in patients with neuroendocrine tumours. Eur J Nucl Med Mol Imaging 34(10):1617–1626

    PubMed  CAS  Google Scholar 

  • Butturini G, Bettini R et al (2006) Predictive factors of efficacy of the somatostatin analogue octreotide as first line therapy for advanced pancreatic endocrine carcinoma. Endocr Relat Cancer 13(4):1213–1221

    PubMed  CAS  Google Scholar 

  • Chatzipantelis P, Salla C et al (2008) Endoscopic ultrasound-guided fine-needle aspiration cytology of pancreatic neuroendocrine tumors: a study of 48 cases. Cancer 114(4):255–262

    PubMed  Google Scholar 

  • Christ E, Wild D et al (2009) Glucagon-like peptide-1 receptor imaging for localization of insulinomas. J Clin Endocrinol Metab 94(11):4398–4405

    PubMed  CAS  Google Scholar 

  • Chung DC, Smith AP et al (1997) A novel pancreatic endocrine tumor suppressor gene locus on chromosome 3p with clinical prognostic implications. J Clin Invest 100(2):404–410

    PubMed Central  PubMed  CAS  Google Scholar 

  • Corleto VD, Annibale B et al (2001) Does the widespread use of proton pump inhibitors mask, complicate and/or delay the diagnosis of Zollinger-Ellison syndrome? Aliment Pharmacol Ther 15(10):1555–1561

    PubMed  CAS  Google Scholar 

  • D’Onofrio M, Mansueto G et al (2004) Neuroendocrine pancreatic tumor: value of contrast enhanced ultrasonography. Abdom Imaging 29(2):246–258

    PubMed  Google Scholar 

  • de Herder WW, Niederle B et al (2006) Well-differentiated pancreatic tumor/carcinoma: insulinoma. Neuroendocrinology 84(3):183–188

    PubMed  Google Scholar 

  • Doppman JL, Chang R et al (1995) Localization of insulinomas to regions of the pancreas by intra-arterial stimulation with calcium. Ann Intern Med 123(4):269–273

    PubMed  CAS  Google Scholar 

  • Drougas JG, Anthony LB et al (1998) Hepatic artery chemoembolization for management of patients with advanced metastatic carcinoid tumors. Am J Surg 175(5):408–412

    PubMed  CAS  Google Scholar 

  • Ehehalt F, Saeger HD et al (2009) Neuroendocrine tumors of the pancreas. Oncologist 14:456–467

    PubMed  CAS  Google Scholar 

  • Ekeblad S, Sundin A et al (2007) Temozolomide as monotherapy is effective in treatment of advanced malignant neuroendocrine tumors. Clin Cancer Res 13(10):2986–2991

    PubMed  CAS  Google Scholar 

  • Eriksson B (2010) New drugs in neuroendocrine tumors: rising of new therapeutic philosophies? Curr Opin Oncol 22(4):381–386

    PubMed  CAS  Google Scholar 

  • Espana-Gomez MN, Velazquez-Fernandez D et al (2009) Pancreatic insulinoma: a surgical experience. World J Surg 33:1966–1970

    PubMed  Google Scholar 

  • Falconi M, Zerbi A et al (2010) Parenchyma-preserving resections for small nonfunctioning pancreatic endocrine tumors. Ann Surg Oncol 17(6):1621–1627

    PubMed  Google Scholar 

  • Falconi M, Bartsch DK et al (2012) ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms of the digestive system: well-differentiated pancreatic non-functioning tumors. Neuroendocrinology 95(2):120–134

    PubMed  CAS  Google Scholar 

  • Fazio N, de Braud F et al (2007) Interferon-alpha and somatostatin analog in patients with gastroenteropancreatic neuroendocrine carcinoma: single agent or combination? Ann Oncol 18(1):13–19

    PubMed  CAS  Google Scholar 

  • Feelders RA, Hofland LJ et al (2010) Medical treatment of cushing’s syndrome: adrenal-blocking drugs and ketoconazole. Neuroendocrinology 92(Suppl 1):111–115

    PubMed  CAS  Google Scholar 

  • Fendrich V, Langer P et al (2007) Management of sporadic and multiple endocrine neoplasia type 1 gastrinomas. Br J Surg 94(11):1331–1341

    PubMed  CAS  Google Scholar 

  • Fernández-Cruz L, Blanco L, Cosa R, Rendón H. (2008) Is laparoscopic resection adequate in patients with neuroendocrine pancreatic tumors? World J Surg 32(5):904–17.

    PubMed  Google Scholar 

  • Frucht H, Howard JM et al (1989) Secretin and calcium provocative tests in the Zollinger-Ellison syndrome. A prospective study. Ann Intern Med 111(9):713–722

    PubMed  CAS  Google Scholar 

  • Garcia-Carbonero R, Capdevila J et al (2010) Incidence, patterns of care and prognostic factors for outcome of gastroenteropancreatic neuroendocrine tumors (GEP-NETs): results from the National Cancer Registry of Spain (RGETNE). Ann Oncol 21(9):1794–1803

    PubMed  CAS  Google Scholar 

  • Gibril F, Schumann M et al (2004) Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore) 83(1):43–83

    Google Scholar 

  • Gillams A, Cassoni A et al (2005) Radiofrequency ablation of neuroendocrine liver metastases: the Middlesex experience. Abdom Imaging 30(4):435–441

    PubMed  CAS  Google Scholar 

  • Gimm O, Konig E et al (2007) Intra-operative quick insulin assay to confirm complete resection of insulinomas guided by selective arterial calcium injection (SACI). Langenbecks Arch Surg 392(6):679–684

    PubMed  Google Scholar 

  • Gola M, Doga M et al (2006) Neuroendocrine tumors secreting growth hormone-releasing hormone: pathophysiological and clinical aspects. Pituitary 9(3):221–229

    PubMed  CAS  Google Scholar 

  • Gupta S, Johnson MM et al (2005) Hepatic arterial embolization and chemoembolization for the treatment of patients with metastatic neuroendocrine tumors: variables affecting response rates and survival. Cancer 104(8):1590–1602

    PubMed  Google Scholar 

  • Halfdanarson TR, Rabe KG et al (2008) Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol 19(10):1727–1733

    PubMed Central  PubMed  CAS  Google Scholar 

  • Haugvik SP, Marangos IP et al (2013) Long-term outcome of laparoscopic surgery for pancreatic neuroendocrine tumors. World J Surg 37(3):582–590

    PubMed  Google Scholar 

  • Hill JS, McPhee JT et al (2009) Pancreatic neuroendocrine tumors: the impact of surgical resection on survival. Cancer 115(4):741–751

    PubMed  Google Scholar 

  • Hoff AO, Vassilopoulou-Sellin R (1998) The role of glucagon administration in the diagnosis and treatment of patients with tumor hypoglycemia. Cancer 82(8):1585–1592

    PubMed  CAS  Google Scholar 

  • Horwhat JD, Paulson EK et al (2006) A randomized comparison of EUS-guided FNA versus CT or US-guided FNA for the evaluation of pancreatic mass lesions. Gastrointest Endosc 63(7):966–975

    PubMed  Google Scholar 

  • Hough DM, Stephens DH et al (1994) Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings. AJR Am J Roentgenol 162(5):1091–1094

    PubMed  CAS  Google Scholar 

  • Ichikawa T, Peterson MS et al (2000) Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection. Radiology 216(1):163–171

    PubMed  CAS  Google Scholar 

  • Jackson JE (2005) Angiography and arterial stimulation venous sampling in the localization of pancreatic neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 19(2):229–239

    PubMed  Google Scholar 

  • Jensen RT, Niederle B et al (2006) Gastrinoma (duodenal and pancreatic). Neuroendocrinology 84(3):173–182

    PubMed  CAS  Google Scholar 

  • Jensen RT, Berna MJ et al (2008) Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer 113(7 Suppl):1807–1843

    PubMed Central  PubMed  Google Scholar 

  • Jensen RT, Cadiot G et al (2012) ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology 95(2):98–119

    PubMed Central  PubMed  CAS  Google Scholar 

  • Joseph S, Wang YZ et al (2011) Neuroendocrine tumors: current recommendations for diagnosis and surgical management. Endocrinol Metab Clin North Am 40(1):205–231, x

    PubMed  Google Scholar 

  • Kaklamanos M, Karoumpalis I et al (2011) Diagnostic accuracy and clinical significance of the fine needle aspiration Ki-67 labelling index in pancreatic endocrine tumours. Endocr Relat Cancer 18(6):L1–L3

    PubMed  Google Scholar 

  • Kalinowski M, Dressler M et al (2009) Selective internal radiotherapy with Yttrium-90 microspheres for hepatic metastatic neuroendocrine tumors: a prospective single center study. Digestion 79(3):137–142

    PubMed  Google Scholar 

  • Kaltsas G, Rockall A et al (2004a) Recent advances in radiological and radionuclide imaging and therapy of neuroendocrine tumours. Eur J Endocrinol 151(1):15–27

    PubMed  CAS  Google Scholar 

  • Kaltsas GA, Besser GM et al (2004b) The diagnosis and medical management of advanced neuroendocrine tumors. Endocr Rev 25(3):458–511

    PubMed  CAS  Google Scholar 

  • Kaltsas G, Androulakis II et al (2010) Paraneoplastic syndromes secondary to neuroendocrine tumours. Endocr Relat Cancer 17(3):R173–R193

    PubMed  Google Scholar 

  • Kanakis G, Kaltsas G et al (2012) Unusual complication of a pancreatic neuroendocrine tumor presenting with malignant hypercalcemia. J Clin Endocrinol Metab 97(4):E627–E631

    PubMed  CAS  Google Scholar 

  • Kao PC, Taylor RL et al (1994) Proinsulin by immunochemiluminometric assay for the diagnosis of insulinoma. J Clin Endocrinol Metab 78(5):1048–1051

    PubMed  CAS  Google Scholar 

  • Kianmanesh R, Sauvanet A et al (2008) Two-step surgery for synchronous bilobar liver metastases from digestive endocrine tumors: a safe approach for radical resection. Ann Surg 247(4):659–665

    PubMed  Google Scholar 

  • Kimura W, Kuroda A et al (1991) Clinical pathology of endocrine tumors of the pancreas. Analysis of autopsy cases. Dig Dis Sci 36(7):933–942

    PubMed  CAS  Google Scholar 

  • Kindmark H, Sundin A et al (2007) Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years. Med Oncol 24(3):330–337

    PubMed  CAS  Google Scholar 

  • Klimstra DS, Modlin IR et al (2010) The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas 39(6):707–712

    PubMed  Google Scholar 

  • Kloppel G, Anlauf M et al (2007a) Endocrine precursor lesions of gastroenteropancreatic neuroendocrine tumors. Endocr Pathol 18(3):150–155

    PubMed  Google Scholar 

  • Kloppel G, Rindi G et al (2007b) Site-specific biology and pathology of gastroenteropancreatic neuroendocrine tumors. Virchows Arch 451(Suppl 1):S9–S27

    PubMed  Google Scholar 

  • Kouvaraki MA, Shapiro SE et al (2006) Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg 30(5):643–653

    PubMed  Google Scholar 

  • Kulke MH, Stuart K et al (2006) Phase II study of temozolomide and thalidomide in patients with metastatic neuroendocrine tumors. J Clin Oncol 24(3):401–406

    PubMed  CAS  Google Scholar 

  • Kulke MH, Bergsland EK et al (2009) Glycemic control in patients with insulinoma treated with everolimus. N Engl J Med 360(2):195–197

    PubMed  CAS  Google Scholar 

  • Kwekkeboom DJ, Mueller-Brand J et al (2005) Overview of results of peptide receptor radionuclide therapy with 3 radiolabeled somatostatin analogs. J Nucl Med 46(Suppl 1):62S–66S

    PubMed  CAS  Google Scholar 

  • Kwekkeboom DJ, de Herder WW et al (2008) Treatment with the radiolabeled somatostatin analog [177 Lu-DOTA 0, Tyr3]octreotate: toxicity, efficacy, and survival. J Clin Oncol 26(13):2124–2130

    PubMed  CAS  Google Scholar 

  • Lamberts SW, Reubi JC et al (1992) Somatostatin receptor imaging in the diagnosis and treatment of neuroendocrine tumors. J Steroid Biochem Mol Biol 43(1–3):185–188

    PubMed  CAS  Google Scholar 

  • Langer P, Wild A et al (2001) Prospective controlled trial of a standardized meal stimulation test in the detection of pancreaticoduodenal endocrine tumours in patients with multiple endocrine neoplasia type 1. Br J Surg 88(10):1403–1407

    PubMed  CAS  Google Scholar 

  • Lehnert T (1998) Liver transplantation for metastatic neuroendocrine carcinoma: an analysis of 103 patients. Transplantation 66(10):1307–1312

    PubMed  CAS  Google Scholar 

  • Levy-Bohbot N, Merle C et al (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

    PubMed  Google Scholar 

  • Ligneau B, Lombard-Bohas C et al (2001) Cystic endocrine tumors of the pancreas: clinical, radiologic, and histopathologic features in 13 cases. Am J Surg Pathol 25(6):752–760

    PubMed  CAS  Google Scholar 

  • Lonovics J, Devitt P et al (1981) Pancreatic polypeptide. A review. Arch Surg 116(10):1256–1264

    PubMed  CAS  Google Scholar 

  • Lorenz K, Dralle H (2007) Surgical treatment of sporadic gastrinoma. Wien Klin Wochenschr 119:597–601

    PubMed  Google Scholar 

  • Madura JA, Cummings OW et al (1997) Nonfunctioning islet cell tumors of the pancreas: a difficult diagnosis but one worth the effort. Am Surg 63(7):573–577, discussion 577–578

    PubMed  CAS  Google Scholar 

  • Malago R, D’Onofrio M et al (2009) Contrast-enhanced sonography of nonfunctioning pancreatic neuroendocrine tumors. AJR Am J Roentgenol 192(2):424–430

    PubMed  Google Scholar 

  • Marotta V, Nuzzo V et al (2012) Limitations of Chromogranin A in clinical practice. Biomarkers 17(2):186–191

    PubMed  CAS  Google Scholar 

  • Massironi S, Conte D et al (2010) Plasma chromogranin A response to octreotide test: prognostic value for clinical outcome in endocrine digestive tumors. Am J Gastroenterol 105(9):2072–2078

    PubMed  CAS  Google Scholar 

  • Maton PN (1993) Use of octreotide acetate for control of symptoms in patients with islet cell tumors. World J Surg 17(4):504–510

    PubMed  CAS  Google Scholar 

  • Maton PN, Gardner JD et al (1986) Cushing’s syndrome in patients with the Zollinger-Ellison syndrome. N Engl J Med 315(1):1–5

    PubMed  CAS  Google Scholar 

  • Matthews BD, Heniford BT et al (2000) Surgical experience with nonfunctioning neuroendocrine tumors of the pancreas. Am Surg 66(12):1116–1122, discussion 1122–1113

    PubMed  CAS  Google Scholar 

  • McLean AM, Fairclough PD (2005) Endoscopic ultrasound in the localisation of pancreatic islet cell tumours. Best Pract Res Clin Endocrinol Metab 19(2):177–193

    PubMed  CAS  Google Scholar 

  • Modlin IM, Oberg K et al (2008) Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol 9(1):61–72

    PubMed  CAS  Google Scholar 

  • Moertel CG, Hanley JA et al (1980) Streptozocin alone compared with streptozocin plus fluorouracil in the treatment of advanced islet-cell carcinoma. N Engl J Med 303(21):1189–1194

    PubMed  CAS  Google Scholar 

  • Moertel CG, Kvols LK et al (1991) Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Evidence of major therapeutic activity in the anaplastic variants of these neoplasms. Cancer 68(2):227–232

    PubMed  CAS  Google Scholar 

  • Moertel CG, Lefkopoulo M et al (1992) Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 326(8):519–523

    PubMed  CAS  Google Scholar 

  • Newell-Price J, Bertagna X et al (2006) Cushing’s syndrome. Lancet 367(9522):1605–1617

    PubMed  CAS  Google Scholar 

  • Nilsson O, Van Cutsem E et al (2006) Poorly differentiated carcinomas of the foregut (gastric, duodenal and pancreatic). Neuroendocrinology 84(3):212–215

    PubMed  CAS  Google Scholar 

  • Nino-Murcia M, Tamm EP et al (2003) Multidetector-row helical CT and advanced postprocessing techniques for the evaluation of pancreatic neoplasms. Abdom Imaging 28(3):366–377

    PubMed  CAS  Google Scholar 

  • Nobels FR, Kwekkeboom DJ et al (1997) Chromogranin A as serum marker for neuroendocrine neoplasia: comparison with neuron-specific enolase and the alpha-subunit of glycoprotein hormones. J Clin Endocrinol Metab 82(8):2622–2628

    PubMed  CAS  Google Scholar 

  • Norton JA, Cromack DT et al (1988) Intraoperative ultrasonographic localization of islet cell tumors. A prospective comparison to palpation. Ann Surg 207(2):160–168

    PubMed Central  PubMed  CAS  Google Scholar 

  • Norton JA, Alexander HR et al (2004) Does the use of routine duodenotomy (DUODX) affect rate of cure, development of liver metastases or survival in patients with Zollinger-Ellison syndrome? Ann Surg 239:617–626

    PubMed Central  PubMed  Google Scholar 

  • Norton JA, Fang TD et al (2006) Surgery for gastrinoma and insulinoma in multiple endocrine neoplasia type 1. J Natl Compr Canc Netw 4(2):148–153

    PubMed  Google Scholar 

  • O’Brien TD, Chejfec G et al (1993) Clinical features of duodenal somatostatinomas. Surgery 114(6):1144–1147

    PubMed  Google Scholar 

  • O’Toole D, Salazar R et al (2006) Rare functioning pancreatic endocrine tumors. Neuroendocrinology 84(3):189–195

    PubMed  Google Scholar 

  • O’Toole D, Grossman A et al (2009) ENETS Consensus Guidelines for the standards of care in neuroendocrine tumors: biochemical markers. Neuroendocrinology 90(2):194–202

    PubMed  Google Scholar 

  • Oberg K (2011) Circulating biomarkers in gastroenteropancreatic neuroendocrine tumours. Endocr Relat Cancer 18(Suppl 1):S17–S25

    PubMed  CAS  Google Scholar 

  • Oberg KE, Reubi JC et al (2010) Role of somatostatins in gastroenteropancreatic neuroendocrine tumor development and therapy. Gastroenterology 139(3):742–753, 753 e741

    PubMed  Google Scholar 

  • Olausson M, Friman S et al (2002) Indications and results of liver transplantation in patients with neuroendocrine tumors. World J Surg 26(8):998–1004

    PubMed  Google Scholar 

  • Orlefors H, Sundin A et al (2005) Whole-body (11)C-5-hydroxytryptophan positron emission tomography as a universal imaging technique for neuroendocrine tumors: comparison with somatostatin receptor scintigraphy and computed tomography. J Clin Endocrinol Metab 90(6):3392–3400

    PubMed  CAS  Google Scholar 

  • Owen NJ, Sohaib SA et al (2001) MRI of pancreatic neuroendocrine tumours. Br J Radiol 74(886):968–973

    PubMed  CAS  Google Scholar 

  • Pape UF, Jann H et al (2008) Prognostic relevance of a novel TNM classification system for upper gastroenteropancreatic neuroendocrine tumors. Cancer 113(2):256–265

    PubMed  Google Scholar 

  • Pasquali C, Rubello D et al (1998) Neuroendocrine tumor imaging: can 18F-fluorodeoxyglucose positron emission tomography detect tumors with poor prognosis and aggressive behavior? World J Surg 22(6):588–592

    PubMed  CAS  Google Scholar 

  • Paulsen SD, Nghiem HV et al (2006) Evaluation of imaging-guided core biopsy of pancreatic masses. AJR Am J Roentgenol 187(3):769–772

    PubMed  Google Scholar 

  • Perez-Montiel MD, Frankel WL et al (2003) Neuroendocrine carcinomas of the pancreas with ‘Rhabdoid’ features. Am J Surg Pathol 27(5):642–649

    PubMed  Google Scholar 

  • Phan GQ, Yeo CJ et al (1998) Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: review of 125 patients. J Gastrointest Surg 2(5):472–482

    PubMed  CAS  Google Scholar 

  • Piani C, Franchi GM et al (2008) Cytological Ki-67 in pancreatic endocrine tumours: an opportunity for pre-operative grading. Endocr Relat Cancer 15(1):175–181

    PubMed  Google Scholar 

  • Portela-Gomes GM, Stridsberg M et al (2000) Expression of the five different somatostatin receptor subtypes in endocrine cells of the pancreas. Appl Immunohistochem Mol Morphol 8(2):126–132

    PubMed  CAS  Google Scholar 

  • Raymond E, Dahan L et al (2011) Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med 364(6):501–513

    PubMed  CAS  Google Scholar 

  • Rehfeld JF, Bardram L et al (2012) Pitfalls in diagnostic gastrin measurements. Clin Chem 58(5):831–836

    PubMed  CAS  Google Scholar 

  • Richards ML, Thompson GB et al (2011) Setting the bar for laparoscopic resection of sporadic insulinoma. World J Surg 35:785–789

    PubMed  Google Scholar 

  • Rigaud G, Missiaglia E et al (2001) High resolution allelotype of nonfunctional pancreatic endocrine tumors: identification of two molecular subgroups with clinical implications. Cancer Res 61(1):285–292

    PubMed  CAS  Google Scholar 

  • Rindi G, Kloppel G (2004) Endocrine tumors of the gut and pancreas tumor biology and classification. Neuroendocrinology 80(Suppl 1):12–15

    PubMed  CAS  Google Scholar 

  • Rindi G, Kloppel G et al (2006) TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 449(4):395–401

    PubMed Central  PubMed  CAS  Google Scholar 

  • Rinke A, Muller HH et al (2009) Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol 27(28):4656–4663

    PubMed  CAS  Google Scholar 

  • Rosenau J, Bahr MJ et al (2002) Ki67, E-cadherin, and p53 as prognostic indicators of long-term outcome after liver transplantation for metastatic neuroendocrine tumors. Transplantation 73(3):386–394

    PubMed  CAS  Google Scholar 

  • Roy PK, Venzon DJ et al (2000) Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore) 79(6):379–411

    CAS  Google Scholar 

  • Sarmiento JM, Heywood G et al (2003) Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival. J Am Coll Surg 197(1):29–37

    PubMed  Google Scholar 

  • Schmid HA, Schoeffter P (2004) Functional activity of the multiligand analog SOM230 at human recombinant somatostatin receptor subtypes supports its usefulness in neuroendocrine tumors. Neuroendocrinology 80(Suppl 1):47–50

    PubMed  CAS  Google Scholar 

  • Service FJ (1995) Hypoglycemic disorders. N Engl J Med 332(17):1144–1152

    PubMed  CAS  Google Scholar 

  • Service FJ, Natt N (2000) The prolonged fast. J Clin Endocrinol Metab 85(11):3973–3974

    PubMed  CAS  Google Scholar 

  • Service FJ, McMahon MM et al (1991) Functioning insulinoma–incidence, recurrence, and long-term survival of patients: a 60-year study. Mayo Clin Proc 66(7):711–719

    PubMed  CAS  Google Scholar 

  • Shibata C, Kakyo M, et al (2012) Criteria for the glucagon provocative test in the diagnosis of gastrinoma. Surg Today 43(11):1281–5

    Google Scholar 

  • Sobin LH, Wittekind C (2002) TNM classification of malignant tumours. Wiley-Liss, New York

    Google Scholar 

  • Soga J, Yakuwa Y (1998a) Glucagonomas/diabetico-dermatogenic syndrome (DDS): a statistical evaluation of 407 reported cases. J Hepatobiliary Pancreat Surg 5(3):312–319

    PubMed  CAS  Google Scholar 

  • Soga J, Yakuwa Y (1998b) Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Cancer Res 17(4):389–400

    PubMed  CAS  Google Scholar 

  • 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

    PubMed  CAS  Google Scholar 

  • Soga J, Yakuwa Y et al (1998) Insulinoma/hypoglycemic syndrome: a statistical evaluation of 1085 reported cases of a Japanese series. J Exp Clin Cancer Res 17(4):379–388

    PubMed  CAS  Google Scholar 

  • Stabile BE, Morrow DJ et al (1984) The gastrinoma triangle: operative implications. Am J Surg 147(1):25–31

    PubMed  CAS  Google Scholar 

  • Steinmuller T, Kianmanesh R et al (2008) Consensus guidelines for the management of patients with liver metastases from digestive (neuro)endocrine tumors: foregut, midgut, hindgut, and unknown primary. Neuroendocrinology 87(1):47–62

    PubMed  Google Scholar 

  • Stridsberg M, Eriksson B et al (2003) A comparison between three commercial kits for chromogranin A measurements. J Endocrinol 177(2):337–341

    PubMed  CAS  Google Scholar 

  • Strosberg JR, Fine RL et al (2010) First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer 117(2):268–275

    PubMed  Google Scholar 

  • Sundin A, Vullierme MP et al (2009) ENETS Consensus Guidelines for the standards of care in neuroendocrine tumors: radiological examinations. Neuroendocrinology 90(2):167–183

    PubMed  CAS  Google Scholar 

  • Thomas T, Kaye PV et al (2009) Efficacy, safety, and predictive factors for a positive yield of EUS-guided Trucut biopsy: a large tertiary referral center experience. Am J Gastroenterol 104(3):584–591

    PubMed  Google Scholar 

  • Tonelli F, Giudici F et al (2011) Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: review of literature. Endocr Pract 17(Suppl 3):33–40

    PubMed  Google Scholar 

  • Travis WD, World Health Organization et al (2004) Pathology and genetics of tumours of the lung, pleura, thymus and heart. Oxford University Press, Oxford (distributor)

    Google Scholar 

  • Triponez F, Goudet P et al (2006) 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 30(5):654–662, discussion 663–654

    PubMed  Google Scholar 

  • Tsolakis AV, Janson ET (2008) Endocrine pancreatic tumors: diagnosis and treatment. Expert Rev Endocrinol Metab 3(2):187–205

    Google Scholar 

  • Vagefi PA, Razo O et al (2007) Evolving patterns in the detection and outcomes of pancreatic neuroendocrine neoplasms: the Massachusetts General Hospital experience from 1977 to 2005. Arch Surg 142(4):347–354

    PubMed  PubMed Central  Google Scholar 

  • Verhoef S, van Diemen-Steenvoorde R et al (1999) Malignant pancreatic tumour within the spectrum of tuberous sclerosis complex in childhood. Eur J Pediatr 158(4):284–287

    PubMed  CAS  Google Scholar 

  • Vezzosi D, Bennet A et al (2005) Octreotide in insulinoma patients: efficacy on hypoglycemia, relationships with Octreoscan scintigraphy and immunostaining with anti-sst2A and anti-sst5 antibodies. Eur J Endocrinol 152(5):757–767

    PubMed  CAS  Google Scholar 

  • Walter T, Chardon L et al (2012) Is the combination of chromogranin A and pancreatic polypeptide serum determinations of interest in the diagnosis and follow-up of gastro-entero-pancreatic neuroendocrine tumours? Eur J Cancer 48(12):1766–1773

    PubMed  CAS  Google Scholar 

  • Yao JC (2007) Neuroendocrine tumors. Molecular targeted therapy for carcinoid and islet-cell carcinoma. Best Pract Res Clin Endocrinol Metab 21(1):163–172

    PubMed  CAS  Google Scholar 

  • Yao JC, Hassan M et al (2008a) One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 26(18):3063–3072

    PubMed  Google Scholar 

  • Yao JC, Phan A et al (2008b) Targeting vascular endothelial growth factor in advanced carcinoid tumor: a random assignment phase II study of depot octreotide with bevacizumab and pegylated interferon alpha-2b. J Clin Oncol 26(8):1316–1323

    PubMed  CAS  Google Scholar 

  • Yao JC, Shah MH et al (2011) Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med 364(6):514–523

    PubMed  CAS  Google Scholar 

  • Zamora V, Cabanne A et al (2010) Immunohistochemical expression of somatostatin receptors in digestive endocrine tumours. Dig Liver Dis 42(3):220–225

    PubMed  CAS  Google Scholar 

  • Zerbi A, Capitanio V et al (2011) Surgical treatment of pancreatic endocrine tumours in Italy: results of a prospective multicentre study of 262 cases. Langenbecks Arch Surg 396(3):313–321

    PubMed  Google Scholar 

  • Zhao J, Moch H et al (2001) Genomic imbalances in the progression of endocrine pancreatic tumors. Genes Chromosomes Cancer 32(4):364–372

    PubMed  CAS  Google Scholar 

  • Zhou C, Dhall D et al (2009) Homozygous P86S mutation of the human glucagon receptor is associated with hyperglucagonemia, alpha cell hyperplasia, and islet cell tumor. Pancreas 38(8):941–946

    PubMed Central  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Medical Sciences, Section of Endocrine Oncology, Uppsala University, 85, Uppsala, Sweden

    Dr. Apostolos Tsolakis

  2. Department of Pathophysiology, University of Athens Medical School, Mikras Asias 75, 11527, Athens, Greece

    Dr. George Kanakis

Authors
  1. Dr. Apostolos Tsolakis
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  2. Dr. George Kanakis
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Correspondence to Apostolos Tsolakis .

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Editors and Affiliations

  1. Södersjukhuset, Karolinska Institutet Dept. Clinical Research & Education, Stockholm, Sweden

    Md. Shahidul Islam

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Tsolakis, A., Kanakis, G. (2014). Pancreatic Neuroendocrine Tumors. In: Islam, M. (eds) Islets of Langerhans, 2. ed.. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-6884-0_50-2

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  • DOI: https://doi.org/10.1007/978-94-007-6884-0_50-2

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