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Pathology and Genetics

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Uncommon Pancreatic Neoplasms

Part of the book series: Updates in Surgery ((UPDATESSURG))

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Abstract

Pancreatic endocrine neoplasms (PanNENs) are epithelial tumors affecting adults between the ages of 40 and 60 [1]. They are usually solitary and sporadic but may be part of hereditary syndromes, including multiple endocrine neoplasia type 1 (MEN1), von Hippel Lindau (VHL), neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC).

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References

  1. Klimstra D, Arnold R, Capella C et al (2010) Neuroendocrine neoplasms of the pancreas. In:Bosman F, Carneiro F, Hruban R, et al (eds) World Health Organization classification of tumors of the digestive system. IARC, Lyon

    Google Scholar 

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

    Article  PubMed  Google Scholar 

  3. Kloppel G, Rindi G, Anlauf M et al (2007) Site-specific biology and pathology of gastroenteropancreatic neuroendocrine tumors. Virchows Arch 451 Suppl 1:S9-27

    Article  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  7. Alexakis N, Neoptolemos JP (2008) Pancreatic neuroendocrine tumours. Best Pract Res Clin Gastroenterol 22:183–205

    Article  PubMed  CAS  Google Scholar 

  8. Hruban RU, Bishop Pitman M, Klimstra DL (2007) Endocrine Neoplasms, in Silverberg SG, Sobin LH (eds): Tumors of the pancreas. Washington, Armed Forces Institute of Pathology

    Google Scholar 

  9. Capelli P, Martignoni G, Pedica F et al (2009) Endocrine neoplasms of the pancreas: pathologic and genetic features. Arch Pathol Lab Med 133:350–364

    PubMed  Google Scholar 

  10. Heitz PU, Komminoth P, Perren A et al (2004) Pancreatic endocrine tumours, in DeLellis RA, Lloyd RV, Heitz PU, et al (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumors of Endocrine Organs. IARC Press, Lyon

    Google Scholar 

  11. Wiedenmann B, Franke WW, Kuhn C, et al (1986) Synaptophysin: a marker protein for neuroendocrine cells and neoplasms. Proc Natl Acad Sci U S A 83:3500–3504

    Article  PubMed  CAS  Google Scholar 

  12. Lloyd RV, Mervak T, Schmidt K et al (1984) Immunohistochemical detection of chromogranin and neuron-specific enolase in pancreatic endocrine neoplasms. Am J Surg Pathol 8:607–14

    Article  PubMed  CAS  Google Scholar 

  13. Bishop AE, Polak JM, Facer P et al (1982) Neuron specific enolase: a common marker for the endocrine cells and innervation of the gut and pancreas. Gastroenterology 83:902–15

    PubMed  CAS  Google Scholar 

  14. Rode J, Dhillon AP, Doran JF et al (1985) PGP 9.5, a new marker for human neuroendocrine tumours. Histopathology 9:147–158

    Article  PubMed  CAS  Google Scholar 

  15. Pelosi G, Bresaola E, Bogina G et al (1996) Endocrine tumors of the pancreas: Ki-67 immunoreactivity on paraffin sections is an independent predictor for malignancy: a comparative study with proliferating-cell nuclear antigen and progesterone receptor protein immunostaining, mitotic index, and other clinicopathologic variables. Hum Pathol 27:1124–1134

    Article  PubMed  CAS  Google Scholar 

  16. Yachida S, Vakiani E, White CM et al (2012) Small cell and large cell neuroendocrine carcinomas of the pancreas are genetically similar and distinct from well-differentiated pancreatic neuroendocrine tumors. Am J Surg Pathol 36:173–184

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  18. Scarpa A, Mantovani W, Capelli P et al (2010) Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients. Mod Pathol 23:824–833

    Article  PubMed  CAS  Google Scholar 

  19. Fischer L, Kleeff J, Esposito I et al (2008) Clinical outcome and long-term survival in 118 consecutive patients with neuroendocrine tumours of the pancreas. Br J Surg 95:627–635

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  21. Barbarotto E, Schmittgen TD, Calin GA (2008): MicroRNAs and cancer: profile, profile, profile. Int J Cancer 122:969–977

    Article  PubMed  CAS  Google Scholar 

  22. Stumpf E, Aalto Y, Hoog A et al (2000) Chromosomal alterations in human pancreatic endocrine tumors. Genes Chromosomes Cancer 29:83–87

    Article  PubMed  CAS  Google Scholar 

  23. Speel EJ, Richter J, Moch H et al (1999) Genetic differences in endocrine pancreatic tumor subtypes detected by comparative genomic hybridization. Am J Pathol 155:1787–1794

    Article  PubMed  CAS  Google Scholar 

  24. Hu W, Feng Z, Modica I et al (2010) Gene Amplifications in Well-Differentiated Pancreatic Neuroendocrine Tumors Inactivate the p53 Pathway. Genes Cancer 1:360–368

    Article  PubMed  CAS  Google Scholar 

  25. Nagano Y, Kim do H, Zhang L et al (2007) Allelic alterations in pancreatic endocrine tumors identified by genome-wide single nucleotide polymorphism analysis. Endocr Relat Cancer 14:483–492

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  27. Missiaglia E, Moore PS, Williamson J et al (2002) Sex chromosome anomalies in pancreatic endocrine tumors. Int J Cancer 98:532–538

    Article  PubMed  CAS  Google Scholar 

  28. Arnold CN, Sosnowski A, Schmitt-Graff A et al (2007) Analysis of molecular pathways in sporadic neuroendocrine tumors of the gastro-entero-pancreatic system. Int J Cancer 120:2157–2164

    Article  PubMed  CAS  Google Scholar 

  29. House MG, Herman JG, Guo MZ et al (2003) Aberrant hypermethylation of tumor suppressor genes in pancreatic endocrine neoplasms. Ann Surg 238:423–431; discussion 431-432

    PubMed  Google Scholar 

  30. Malpeli G, Amato E, Dandrea M et al (2011) Methylation-associated down-regulation of RASSF1A and up-regulation of RASSF1C in pancreatic endocrine tumors. BMC Cancer 11:351

    Article  PubMed  CAS  Google Scholar 

  31. Corbo V, Beghelli S, Bersani S et al (2012) Pancreatic endocrine tumours: mutational and immunohistochemical survey of protein kinases reveals alterations in targetable kinases in cancer cell lines and rare primaries. Ann Oncol 23:127–134

    Article  PubMed  CAS  Google Scholar 

  32. Jiao Y, Shi C, Edil BH et al (2011) DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science 331:1199–1203

    Article  PubMed  CAS  Google Scholar 

  33. Moore PS, Missiaglia E, Antonello D et al (2001) Role of disease-causing genes in sporadic pancreatic endocrine tumors: MEN1 and VHL. Genes Chromosomes Cancer 32:177–181

    Article  PubMed  CAS  Google Scholar 

  34. Corbo V, Dalai I, Scardoni M et al (2010) MEN1 in pancreatic endocrine tumors: analysis of gene and protein status in 169 sporadic neoplasms reveals alterations in the vast majority of cases. Endocr Relat Cancer 17:771–783

    Article  PubMed  CAS  Google Scholar 

  35. Heaphy CM, de Wilde RF, Jiao Y et al (2011) Altered telomeres in tumors with ATRX and DAXX mutations. Science 333:425

    Article  PubMed  CAS  Google Scholar 

  36. Missiaglia E, Dalai I, Barbi S et al (2010) Pancreatic endocrine tumors: expression profiling evidences a role for AKT-mTOR pathway. J Clin Oncol 28:245–255

    Article  PubMed  CAS  Google Scholar 

  37. Roldo C, Missiaglia E, Hagan JP et al (2006) MicroRNA expression abnormalities in pancreatic endocrine and acinar tumors are associated with distinctive pathologic features and clinical behavior. J Clin Oncol 24:4677–4684

    Article  PubMed  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pathology and Diagnostics, Pathology Unit, “G.B. Rossi” University Hospital, Verona, Italy

    Aldo Scarpa

  2. ARC-NET Research Centre, University of Verona, Verona, Italy

    Aldo Scarpa

Authors
  1. Aldo Scarpa
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  2. Vincenzo Corbo
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Corresponding authors

Correspondence to Massimo Falconi or Aldo Scarpa .

Editor information

Editors and Affiliations

  1. Department of Surgery and Oncology General Surgery Unit, Pancreas Center, “G.B. Rossi” University Hospital, Verona, Italy

    Paolo Pederzoli  & Claudio Bassi  & 

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Scarpa, A., Corbo, V. (2013). Pathology and Genetics. In: Pederzoli, P., Bassi, C. (eds) Uncommon Pancreatic Neoplasms. Updates in Surgery. Springer, Milano. https://doi.org/10.1007/978-88-470-2673-5_8

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  • DOI: https://doi.org/10.1007/978-88-470-2673-5_8

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-2672-8

  • Online ISBN: 978-88-470-2673-5

  • eBook Packages: MedicineMedicine (R0)

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