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Neuroendocrine Tumorigenesis

  • Nadine Gantenbein
  • Johannes HaybaeckEmail author
Chapter
  • 551 Downloads

Abstract

In the nineteenth century, T. Langhans, O. Lubarsch, and W. B. Ranson were the first to report on tumors of the small bowel which were not successfully characterized at that time. In September 1907, Siegfried Oberndorfer described lesions of the intestine and termed them “carcinoid.” His nomenclature of neuroendocrine tumors is still valid today [1]. These tumors are rare neoplasms, and about 0.46% of all malignant diseases and 2.5–5 cases per 100,000 individuals per year are neuroendocrine tumors (NETs) [2].

Keywords

Pathway Member Gastroenteropancreatic Neuroendocrine Tumor Integral Membrane Glycoprotein Balance Therapy Acid Nuclear Protein 
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.
    Modlin IM, Shapiro MD, Kidd M. Siegfried oberndorfer: origins and perspectives of carcinoid tumors. Hum Pathol. 2004;35(12):1440–51.CrossRefPubMedGoogle Scholar
  2. 2.
    Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97(4):934–59.CrossRefPubMedGoogle Scholar
  3. 3.
    Solcia E, Klöppel G, Sobin LH. Histological typing of endocrine tumours. 2nd ed. WHO, World Health Organization, International Histological Classification of Tumours; 2000.Google Scholar
  4. 4.
    Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, Abdalla EK, Fleming JB, Vauthey JN, Rashid A, Evans DB. One hundred years after ‘carcinoid’: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26(18):3063–72.CrossRefPubMedGoogle Scholar
  5. 5.
    Niederle MB, Hackl M, Kaserer K, Niederle B. GEPNET: the current incidence and staging based on the WHO and ENETS classification: an analysis based on prospectively collected parameters. Endocr Relat Cancer. 2010;17(4):909–18.CrossRefPubMedGoogle Scholar
  6. 6.
    Klöppel G. Tumour biology and histopathology of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007;21(1):15–31.CrossRefPubMedGoogle Scholar
  7. 7.
    Adler DG. Neuroendocrine tumors: review and clinical update. Hosp Physician. 2007:12–21.Google Scholar
  8. 8.
    Nikou GC, Marinou K, Thomakos P, Papageorgiou D, Sanzanidis V, Nikolaou P, Kosmidis C, Moulakakis A, Mallas E. Chromogranin a levels in diagnosis, treatment and follow-up of 42 patients with non-functioning pancreatic endocrine tumours. Pancreatology. 2008;8(4–5):510–9.CrossRefPubMedGoogle Scholar
  9. 9.
    Marx SJ. Molecular genetics of multiple endocrine neoplasia types 1 and 2. Nat Rev Cancer. 2005;5(5):367–75.CrossRefPubMedGoogle Scholar
  10. 10.
    DeLellis RA. The neuroendocrine system and its tumors: an overview. Am J Clin Pathol. 2001;115(Suppl. 1):5–16.Google Scholar
  11. 11.
    Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, Caplin M, Delle Fave G, Kaltsas GA, Krenning EP, Moss SF, Nilsson O, Rindi G, Salazar R, Ruszniewski P, Sundin A. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9(1):61–72.CrossRefPubMedGoogle Scholar
  12. 12.
    Pfragner R, Behmel A, Hoger H, Beham A, Ingolic E, Stelzer I, Svejda B, Moser VA, Obenauf AC, Siegl V, Haas O, Niederle B. Establishment and characterization of three novel cell lines - P-STS, L-STS, H-STS - derived from a human metastatic midgut carcinoid. Anticancer Res. 2009;29(6):1951–61.PubMedGoogle Scholar
  13. 13.
    Bajetta E, Ferrari L, Martinetti A, Celio L, Procopio G, Artale S, Zilembo N, Di Bartolomeo M, Seregni E, Bombardieri E. Chromogranin A, neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors. Cancer. 1999;86:858–65.CrossRefPubMedGoogle Scholar
  14. 14.
    Jiao Y, Shi C, Edil BH, De Wilde RF, Klimstra DS, Maitra A, Schulick RD, Tang LH, Wolfgang CL, Choti A, Velculescu VE, Jr LAD, Vogelstein B, Kinzler KW, Hruban RH, Papadopoulos N. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011;331(6021):1199–203.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Agarwal SK, Kester MB, Debelenko LV, Heppner C, Emmert-Buck MR, Skarulis MC, Doppman JL, Kim YS, Lubensky IA, Zhuang Z, Green JS, Guru SC, Manickam P, Olufemi SE, Liotta LA, Chandrasekharappa SC, Collins FS, Spiegel AM, Burns AL, Marx SJ. Germline mutations of the MEN1 gene in familial multiple endocrine neoplasia type 1 and related states. Hum Mol Genet. 1997;6(7):1169–75.CrossRefPubMedGoogle Scholar
  16. 16.
    Pieterman CRC, Conemans EB, Dreijerink KMA, De Laat JM, Timmers HTM. Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis. Endocr Relat Cancer. 2014;21(3):R121–42.CrossRefPubMedGoogle Scholar
  17. 17.
    Marinoni I, Kurrer AS, Vassella E, Dettmer M, Rudolph T, Banz V, Hunger F, Pasquinelli S, Speel EJ, Perren A. Loss of DAXX and ATRX are associated with chromosome instability and reduced survival of patients with pancreatic neuroendocrine tumors. Gastroenterology. 2014;146(2):453–460.e5.CrossRefPubMedGoogle Scholar
  18. 18.
    Lemos MC, Thakker RV. Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene. Hum Mutat. 2008;29(1):22–32.CrossRefPubMedGoogle Scholar
  19. 19.
    Asa SL. Pancreatic endocrine tumors. Mod Pathol. 2011;24(Suppl. 2):S66–77.CrossRefPubMedGoogle Scholar
  20. 20.
    Larsson C, Skogseid B, Oberg K, Nakamura Y, Nordenskjöld M. Multiple endocrine neoplasia type 1 gene maps to chromosome 11 and is lost in insulinoma. Nature. 1988;332(6159):85–7.CrossRefPubMedGoogle Scholar
  21. 21.
    Yaguchi H, Ohkura N, Takahashi M, Nagamura Y, Kitabayashi I, Tsukada T. Menin missense mutants associated with multiple endocrine neoplasia type 1 are rapidly degraded via the ubiquitin-proteasome pathway. Mol Cell Biol. 2004;24(15):6569–80.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Cives M, Kunz PL, Morse B, Coppola D, Schell MJ, Campos T, Nguyen PT, Nandoskar P, Khandelwal V, Strosberg JR. Phase II clinical trial of pasireotide long-acting repeatable in patients with metastatic neuroendocrine tumors. Endocr Relat Cancer. 2015;22(1):1–9.CrossRefPubMedGoogle Scholar
  23. 23.
    Missiaglia E, Dalai I, Barbi S, Beghelli S, Falconi M, Della Peruta M, Piemonti L, Capurso G, Di Florio A, Delle Fave G, Pederzoli P, Croce CM, Scarpa A. Pancreatic endocrine tumors: expression profiling evidences a role for AKT-mTOR pathway. J Clin Oncol. 2010;28(2):245–55.CrossRefPubMedGoogle Scholar
  24. 24.
    Perren A, Anlauf M, Henopp T, Rudolph T, Schmitt A, Raffel A, Gimm O, Weihe E, Knoefel WT, Dralle H, Heitz PU, Komminoth P, Klöppel G. Multiple endocrine neoplasia type 1 (MEN1): loss of one MEN1 allele in tumors and monohormonal endocrine cell clusters but not in islet hyperplasia of the pancreas. J Clin Endocrinol Metab. 2007;92(3):1118–28.CrossRefPubMedGoogle Scholar
  25. 25.
    Neary PC, Redmond PH, Houghton T, Watson GR, Bouchier-Hayes D. Carcinoid disease: review of the literature. Dis Colon Rectum. 1997;40(3):349–62.CrossRefPubMedGoogle Scholar
  26. 26.
    Modlin IM, Moss SF, Gustafsson BI, Lawrence B, Schimmack S, Kidd M. The archaic distinction between functioning and nonfunctioning neuroendocrine neoplasms is no longer clinically relevant. Langenbeck’s Arch Surg. 2011;396:1145–56.CrossRefGoogle Scholar
  27. 27.
    Panzuto F, Severi C, Cannizzaro R, Falconi M, Angeletti S, Pasquali A, Corleto VD, Annibale B, Buonadonna A, Pederzoli P, Delle Fave G. Utility of combined use of plasma levels of chromogranin A and pancreatic polypeptide in the diagnosis of gastrointestinal and pancreatic endocrine tumors. J Endocrinol Investig. 2004;27(1):6–11.CrossRefGoogle Scholar
  28. 28.
    Bumming P, Nilsson O, Ahlman H, Welbencer A, Andersson MK, Sjolund K, Nilsson B, Bümming P, Sjölund K. Gastrointestinal stromal tumors regularly express synaptic vesicle proteins: evidence of a neuroendocrine phenotype. Endocr Relat Cancer. 2007;14(3):853–63.CrossRefPubMedGoogle Scholar
  29. 29.
    Andrén-Sandberg A. Pancreatic endocrine tumors. N Am J Med Sci. 2011;3(4):164–6.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Oberg K, Knigge U, Kwekkeboom D, Perren A. Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012;23(Suppl. 7):vii124–30.PubMedGoogle Scholar
  31. 31.
    Modlin IM, Moss SF, Oberg K, Padbury R, Hicks RJ, Gustafsson BI, Wright NA, Kidd M. Gastrointestinal neuroendocrine (carcinoid) tumours: Current diagnosis and management. Med J Aust. 2010;193(1):46–52.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of PathologyMedical University of GrazGrazAustria
  2. 2.Department of PathologyMedical Faculty, Otto von Guericke University MagdeburgMagdeburgGermany
  3. 3.Institute of PathologyMedical University of GrazGrazAustria

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