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Epidemiology of Neuroendocrine Tumors

Chapter

Abstract

Neuroendocrine tumors (NETs) arise from neuroendocrine cells in the endocrine and central nervous systems. NETs are responsible from approximately 0.5 % of all cancers. The incidence has gradually increased during the last three decades. The crude incidence is about 0.2/100,000. It increases with age and peaks between 50 and 70 years. Due to slowly growing nature of NETs, its prevalence is increasing. The prevalence has been estimated to be 35/100,000/year. Most of NETs are diagnosed at advanced stages. Gastrointestinal tract is the most common location and is responsible for two-thirds of NETs. Most of them were diagnosed at the localized stage. However, NETs originating in pancreas tend to be aggressive and about 60 % of these tumors are malignant at the time of diagnosis. The prognosis of NETs associates with their location, functional status, differentiation, and initial stages. The best survival rates are observed in patients with NETs arising in the rectum and appendix.

Keywords

Carcinoid Tumor Multiple Endocrine Neoplasia Type Menopausal Hormone Therapy Atypical Carcinoid Pancreatic NETs 
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.
    Taal BG, Visser O (2004) Epidemiology of neuroendocrine tumours. Neuroendocrinology 80(Suppl 1):3–7CrossRefPubMedGoogle Scholar
  2. 2.
    Yao JC, Hassan M, Phan A et al (2008) One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 26:3063–3072CrossRefPubMedGoogle Scholar
  3. 3.
    Modlin IM, Moss SF, Chung DC, Jensen RT, Snyderwine E (2008) Priorities for improving the management of gastroenteropancreatic neuroendocrine tumors. J Natl Cancer Inst 100:1282–1289CrossRefPubMedCentralPubMedGoogle Scholar
  4. 4.
    Modlin IM, Oberg K, Chung DC et al (2008) Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol 9:61–72CrossRefPubMedGoogle Scholar
  5. 5.
    Mamikunian G, Vinik AI, O’Dorisio TM, Woltering EA, Go VLW (2009) Diagnosing and treating gastroenteropancreatic tumors, including ICD-9 codes. In: Neuroendocrine tumors: a comprehensive guide to diagnosis and management, 4th edn. Inter Science Institute, Inglewood,pp 1–43Google Scholar
  6. 6.
    Tsikitis VL, Wertheim BC, Guerrero MA (2012) Trends of incidence and survival of gastrointestinal neuroendocrine tumors in the United States: a seer analysis. J Cancer 3:292–302CrossRefPubMedCentralPubMedGoogle Scholar
  7. 7.
    Garcia-Carbonero R, Capdevila J, Crespo-Herrero G 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 21:1794–1803CrossRefPubMedGoogle Scholar
  8. 8.
    Fraenkel M, Kim M, Faggiano A, de Herder WW, Valk GD, Knowledge NETwork (2014) Incidence of gastroenteropancreatic neuroendocrine tumours: a systematic review of the literature. Endocr Relat Cancer 21:R153–R163CrossRefPubMedGoogle Scholar
  9. 9.
    Yalcin S, Glasberg S, Abali H et al (2014) Gastroenteropancreatic Neuroendocrine Tumors (GEPNET) Registry: update from an International Collaboration. Ann Oncol Suppl 4;Abstract number: 1141PDGoogle Scholar
  10. 10.
    Korse CM, Taal BG, van Velthuysen ML, Visser O (2013) Incidence and survival of neuroendocrine tumours in the Netherlands according to histological grade: experience of two decades of cancer registry. Eur J Cancer 49:1975–1983CrossRefPubMedGoogle Scholar
  11. 11.
    Modlin IM, Lye KD, Kidd M (2003) A 5-decade analysis of 13,715 carcinoid tumors. Cancer 97:934–959CrossRefPubMedGoogle Scholar
  12. 12.
    Schimmack S, Svejda B, Lawrence B, Kidd M, Modlin IM (2011) The diversity and commonalities of gastroenteropancreatic neuroendocrine tumors. Langenbecks Arch Surg 396:273–298CrossRefPubMedGoogle Scholar
  13. 13.
    Lawrence B, Gustafsson BI, Chan A, Svejda B, Kidd M, Modlin IM (2011) The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin North Am 40:1–18CrossRefPubMedGoogle Scholar
  14. 14.
    Ellis L, Shale MJ, Coleman MP (2010) Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenterol 105:2563–2569CrossRefPubMedGoogle Scholar
  15. 15.
    Cho MY, Kim JM, Sohn JH et al (2012) Current trends of the incidence and pathological diagnosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in Korea 2000–2009: multicenter study. Cancer Res Treat 44:157–165CrossRefPubMedCentralPubMedGoogle Scholar
  16. 16.
    Fahy BN, Tang LH, Klimstra D (2007) Carcinoid of the rectum risk stratification (CaRRS): a strategy for preoperative outcome assessment. Ann Surg Oncol 14:396–404CrossRefPubMedGoogle Scholar
  17. 17.
    Hirano Y, Hara T, Nozawa H et al (2008) Combined choriocarcinoma, neuroendocrine cell carcinoma and tubular adenocarcinoma in the stomach. World J Gastroenterol 14:3269–3272CrossRefPubMedCentralPubMedGoogle Scholar
  18. 18.
    Niederle MB, Hackl M, Kaserer K, Niederle B (2010) Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters. Endocr Relat Cancer 17:909–918CrossRefPubMedGoogle Scholar
  19. 19.
    Chen WF, Zhou PH, Li QL, Xu MD, Yao LQ (2012) Clinical impact of endoscopic submucosal dissection for gastric neuroendocrine tumors: a retrospective study from mainland China. Scientific World J 2012:869769. doi: 10.1100/2012/869769 Google Scholar
  20. 20.
    Rindi G, Bordi C, Rappel S et al (1996) Gastric carcinoids and neuroendocrine carcinomas: pathogenesis, pathology, and behavior. World J Surg 20:168–172CrossRefPubMedGoogle Scholar
  21. 21.
    Borch K, Ahrén B, Ahlman H et al (2005) Gastric carcinoids: biologic behavior and prognosis after differentiated treatment in relation to type. Ann Surg 242:64–73CrossRefPubMedCentralPubMedGoogle Scholar
  22. 22.
    Akerström G, Hellman P (2007) Surgery on neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 21:87–109CrossRefPubMedGoogle Scholar
  23. 23.
    Thompson NW (1998) Management of pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1. Surg Clin N Am 7:881–891Google Scholar
  24. 24.
    Christ E, Wild D, Forrer F, Brändle M, Sahli R, Clerici T, Gloor B, Martius F, Maecke H, Reubi JC (2009) Glucagon-like peptide-1 receptor imaging for localization of insulinomas. J Clin Endocrinol Metab 94:4398–4405CrossRefPubMedGoogle Scholar
  25. 25.
    Klimstra DS, Arnold R, Capella C et al (2010) Neuroendocrine neoplasms of the pancreas. In: Bosman FT, Carneiro F, Hruban RH, Theise ND (eds) WHO classification of tumours of the digestive system. IARC Press, Lyon, pp 322–326Google Scholar
  26. 26.
    Halfdanarson R, Rubin J, Farnell MB, Grant CS, Petersen GM (2008) Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors. Endocr Relat Cancer 15:409–427CrossRefPubMedCentralPubMedGoogle Scholar
  27. 27.
    Metz DC, Jensen RT (2008) Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology 135:1469–1492CrossRefPubMedCentralPubMedGoogle Scholar
  28. 28.
    Valente M, Catena L, Milione M, Pusceddu S, Formisano B, Bajetta E (2010) Common diagnostic challenges in the histopathologic diagnosis of neuroendocrine lung tumors: A case report. Case Rep Oncol 3:202–207CrossRefPubMedCentralPubMedGoogle Scholar
  29. 29.
    Rekhtmann N (2010) Neuroendocrine tumors of the lung. Arch Pathol Lab Med 134:1628–1638Google Scholar
  30. 30.
    Bilimoria KY, Talamonti MS, Tomlinson JS et al (2008) Prognostic score predicting survival after resection of pancreatic neuroendocrine tumors: analysis of 3,851 patients. Ann Surg 247:490–500CrossRefPubMedGoogle Scholar
  31. 31.
    Bettini R, Boninsegna L, Mantovani W et al (2008) Prognostic factors at diagnosis and value of WHO classification in a mono-institutional series of 180 non-functioning pancreatic endocrine tumours. Ann Oncol 19:903–908CrossRefPubMedGoogle Scholar
  32. 32.
    Madeira I, Terris B, Voss M et al (1998) Prognostic factors in patients with endocrine tumours of the duodenopancreatic area. Gut 43:422–427CrossRefPubMedCentralPubMedGoogle Scholar
  33. 33.
    Jarufe NP, Coldham C, Orug T et al (2005) Neuroendocrine tumours of the pancreas: predictors of survival after surgical treatment. Dig Surg 22:157–162CrossRefPubMedGoogle Scholar
  34. 34.
    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 23:824–833CrossRefPubMedGoogle Scholar
  35. 35.
    Mignon M (2000) Natural history of neuroendocrine enteropancreatic tumors. Digestion 62(suppl 1):51–58CrossRefPubMedGoogle Scholar
  36. 36.
    Landry CS, Brock G, Scoggins CR, McMasters KM, Martin RC 2nd (2008) Proposed staging system for colon carcinoid tumors based on an analysis of 2,459 patients. J Am Coll Surg 207:874–881CrossRefPubMedGoogle Scholar
  37. 37.
    Janson ET, Holmberg L, Stridsberg M et al (1997) Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center. Ann Oncol 8:685–690CrossRefPubMedGoogle Scholar
  38. 38.
    Shebani KO, Souba WW, Finkelstein DM et al (1999) Prognosis and survival in patients with gastrointestinal tract carcinoid tumors. Ann Surg 229:815–823CrossRefPubMedCentralPubMedGoogle Scholar
  39. 39.
    Pape UF, Böhmig M, Berndt U, Tiling N, Wiedenmann B, Plöckinger U (2004) Survival and clinical outcome of patients with neuroendocrine tumors of the gastroenteropancreatic tract in a German referral center. Ann N Y Acad Sci 1014:222–233CrossRefPubMedGoogle Scholar
  40. 40.
    Quaedvlieg PF, Visser O, Lamers CB, Janssen-Heijen ML, Taal BG (2001) Epidemiology and survival in patients with carcinoid disease in The Netherlands. An epidemiological study with 2391 patients. Ann Oncol 12:1295–1300CrossRefPubMedGoogle Scholar
  41. 41.
    Hoej LB, Nykjær KM, Gronbaek H (2012) Incidence and survival of patients with small intestinal neuroendocrine tumours in a Danish NET center. Scientific World Journal 2012:206350. doi: 10.1100/2012/206350 CrossRefPubMedCentralPubMedGoogle Scholar
  42. 42.
    Yalcin S (2011) Advances in the systemic treatment of pancreatic neuroendocrine tumors. Cancer Treat Rev 37:127–132CrossRefPubMedGoogle Scholar
  43. 43.
    Ellison TA, Edil BH (2012) The current management of pancreatic neuroendocrine tumors. Adv Surg 46:283–296CrossRefPubMedGoogle Scholar
  44. 44.
    Modlin IM, Champaneria MC, Chan AK, Kidd M (2007) A three-decade analysis of 3,911 small intestinal neuroendocrine tumors: the rapid pace of no progress. Am J Gastroenterol 102:1464–1473CrossRefPubMedGoogle Scholar
  45. 45.
    Caldarella A, Crocetti E, Paci E (2011) Distribution, incidence, and prognosis in neuroendocrine tumors: a population based study from a cancer registry. Pathol Oncol Res 17:759–763CrossRefPubMedGoogle Scholar
  46. 46.
    Lepage C, Bouvier AM, Manfredi S, Dancourt V, Faivre J (2006) Incidence and management of primary malignant small bowel cancers: a well-defined French population study. Am J Gastroenterol 101:2826–2832CrossRefPubMedGoogle Scholar
  47. 47.
    Kulke HM, Anthony LB, Bushnell DL et al (2010) Well-differentiated neuroendocrine tumors of the stomach and pancreas. NANETS guidelines. Pancreas 39:735–752CrossRefPubMedCentralPubMedGoogle Scholar
  48. 48.
    Hauso O, Gustafsson BI, Kidd M et al (2008) Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer 113:2655–2664CrossRefPubMedGoogle Scholar
  49. 49.
    Yao JC, Eisner MP, Leary C et al (2007) Population-based study of islet cell carcinoma. Ann Surg Oncol 14:3492–3500CrossRefPubMedCentralPubMedGoogle Scholar
  50. 50.
    Haselkorn T, Whittemore AS, Lilienfeld DE (2005) Incidence of small bowel cancer in the United States and worldwide: geographic, temporal, and racial differences. Cancer Causes Control 16:781–787CrossRefPubMedGoogle Scholar
  51. 51.
    Faggiano A, Ferolla P, Grimaldi F et al (2012) Natural history of gastro-entero-pancreatic and thoracic neuroendocrine tumors. Data from a large prospective and retrospective Italian Epidemiological study: the NET Management Study. J Endocrinol Invest 35:817–823PubMedGoogle Scholar
  52. 52.
    Ploeckinger U, Kloeppel G, Wiedenmann B, Lohmann R (2009) The German NET-registry: an audit on the diagnosis and therapy of neuroendocrine tumors. Neuroendocrinology 90:349–363CrossRefPubMedGoogle Scholar
  53. 53.
    Bogacka B, Marlicz W, Białek A et al (2009) Trends in colorectal neuroendocrine tumors: a 10 years review. Gut 58:A296Google Scholar
  54. 54.
    Kamiński MF, Polkowski M, Reguła J et al (2007) Prevalence and endoscopic features of rectal neuroendocrine tumours among 50 148 participants of the Polish Colorectal-Cancer Screening Programme. Gut 56:A310CrossRefGoogle Scholar
  55. 55.
    Scherübl H, Streller B, Stabenow R et al (2013) Clinically detected gastroenteropancreatic neuroendocrine tumors are on the rise: epidemiological changes in Germany. World J Gastroenterol 19:9012–9019CrossRefPubMedCentralPubMedGoogle Scholar
  56. 56.
    Soga J (2005) Early-stage carcinoids of the gastrointestinal tract. Cancer 103:1587–1595CrossRefPubMedGoogle Scholar
  57. 57.
    Hemminki K, Li X (2001) Incidence trends and risk factors of carcinoid tumors: a nationwide epidemiologic study from Sweden. Cancer 92:2204–2210CrossRefPubMedGoogle Scholar
  58. 58.
    Levi F, Te VC, Randimbison L, Rindi G, La Vecchia C (2000) Epidemiology of carcinoid neoplasms in Vaud, Switzerland, 1974–97. Br J Cancer 83:952–955CrossRefPubMedCentralPubMedGoogle Scholar
  59. 59.
    Godwin JD 2nd (1975) Carcinoid tumors. An analysis of 2,837 cases. Cancer 36:560–569CrossRefPubMedGoogle Scholar
  60. 60.
    Maher ER, Neumann HP, Richard S (2011) von Hippel-Lindau disease: a clinical and scientific review. Eur J Hum Genet 19:617–623CrossRefPubMedCentralPubMedGoogle Scholar
  61. 61.
    Thakker RV, Newey PJ, Walls GV et al (2012) Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab 97:2990–3011CrossRefPubMedGoogle Scholar
  62. 62.
    Jonkers YM, Ramaekers FC, Speel EJ (2007) Molecular alterations during insulinoma tumorigenesis. Biochim Biophys Acta 1775:313–332PubMedGoogle Scholar
  63. 63.
    Babovic-Vuksanovic D, Constantinou CL, Rubin J, Rowland CM, Schaid DJ, Karnes PS (1999) Familial occurrence of carcinoid tumors and association with other malignant neoplasms. Cancer Epidemiol Biomarkers Prev 8:715–719PubMedGoogle Scholar
  64. 64.
    Pieterman CR, Vriens MR, Dreijerink KM, van der Luijt RB, Valk GD (2011) Care for patients with multiple endocrine neoplasia type 1: the current evidence base. Fam Cancer 10:157–171CrossRefPubMedGoogle Scholar
  65. 65.
    Kaerlev L, Teglbjaerg PS, Sabroe S et al (2002) Occupational risk factors for small bowel carcinoid tumor: a European population based case-control study. J Occup Environ Med 44:516–522CrossRefPubMedGoogle Scholar
  66. 66.
    Cross AJ, Hollenbeck AR, Park Y (2013) A large prospective study of risk factors for adenocarcinomas and malignant carcinoid tumors of the small intestine. Cancer Causes Control 24:1737–1746CrossRefPubMedCentralPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Preventive OncologyHacettepe University Cancer InstituteSıhhiye-AnkaraTurkey

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