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Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  • Neuroendocrine Neoplasms (NS Reed, Section Editor)
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Abstract

Purpose of Review

The purpose of our review is to explore global epidemiologic trends of gastroenteropancreatic (GEP) neuroendocrine tumors (NETs). Specifically, we sought to examine whether there are differences in incidence, prevalence, distribution (by primary tumor site, tumor grade, tumor stage at presentation), and overall survival of GEP NETs between different regions of the world.

Recent Findings

GEP NET incidence rates are rising steadily in North America, Asia, and Europe, though this rise appears to be most profound in North America. The distribution of GEP NETs differs regionally as in North America small intestinal and rectal NETs are most prevalent, in Asia rectal and pancreatic NETs are most prevalent, and in Europe small intestinal and pancreatic NETs are most prevalent. Overall survival for patients with GEP NETs appears to be improving with time.

Summary

Some of the global increase in GEP NET incidence can be explained by increased health care utilization. This factor alone, however, does not explain the rise completely. Population-based studies utilizing uniform data collection instruments and a standard pathologic grading system are needed to identify other factors which may be contributing to this phenomenon.

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References

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  1. Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares J, et al. 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.

    Article  Google Scholar 

  2. Sorbye H, Strosberg J, Baudin E, Klimstra D, Yao J. Gastroenteropancreatic high-grade neuroendocrine carcinoma. Cancer. 2014;120(18):2814–23.

    Article  CAS  Google Scholar 

  3. Leoncini E, Boffetta P, Shafir M, Aleksovska K, Boccia S, Rindi G. Increased incidence trend of low-grade and high-grade neuroendocrine neoplasms. Endocrine. 2017;58:368–79.

    Article  CAS  Google Scholar 

  4. Chauhan A, Kohn E, Del Rivero J. Neuroendocrine tumors-less well known, often misunderstood, and rapidly growing in incidence. JAMA Oncol. 2020;6(1):21–2 An important recent publication highlighting the disease prevalence of gastrointestinal NETs and further all NETs in the United States. This publication suggests the public health importance of NETs.

    Article  Google Scholar 

  5. Cives M, Strosberg J. Gastroenteropancreatic neuroendocrine tumors. CA Cancer J Clin. 2018;68(6):471–87.

    Article  Google Scholar 

  6. Dasari A, Shen C, Halperin D, Zhao B, Zhou S, Xu Y, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017;3(10):1335–42 The largest published SEER database query which led to crucial insights about the rising incidence of NETs and GEP NETs and improved OS of NET patients over time.

    Article  Google Scholar 

  7. Dasari A, Mehta K, Sorbye H, Yao J. Gastroenteropancreatic high-grade neuroendocrine carcinomas: a SEER database analysis of 6291 cases. Cancer. 2018;124(4):807–15 One of the rare publications assessing GEP NEC epidemiology. This analysis demonstrates the increasing incidence of GEP NECs however also does contextualize their relative rarity compared to GEP NETs.

    Article  Google Scholar 

  8. Lee M, Harris C, Baeg K, Aronson A, Wisnivesky J, Kim M. Incidence trends of gastroenteropancreatic neuroendocrine tumors in the United States. Clin Gastroenterol Hepatol. 2019;17(11):2212–7.

    Article  Google Scholar 

  9. Lawrence B, Gustafsson B, Chan A, Svejda B, Kidd M, Modlin I, et al. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endorinol Metab Clin North Am. 2011;40(1):1–18.

    Article  Google Scholar 

  10. Shen C, Gu D, Zhou S, Xu Y, Sarshekeh A, Halperin D, et al. Racial differences in the incidence and survival of patients with neuroendocrine tumors. Pancreas. 2019;48(10):1373–9 A rare epidemiologic study which reported on the influence of race and GEP NET incidence by disease stage at diagnosis. This study suggests that Black patients may be most likely to present with metastatic disease compared to patients of other racial backgrounds.

    Article  Google Scholar 

  11. Hallet J, Law C, Cukier M, Saskin R, Liu N, Singh S. Exploring the rising incidence of neuroendocrine tumors: A population-based analysis of epidemiology, metastatic presentation, and outcomes. Cancer. 2015;121(4):589–97.

    Article  Google Scholar 

  12. Palepu J, Shrikhande S, Bhaduri D, Shah R, Sirohi B, Chhabra V, et al. Trends in diagnosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in India: a report of multicenter data from a web-based registry. Indian J Gastroenterol. 2017;36(6):445–51 One of the initial publications reporting on GEP NET epidemiology from India. This report demonstrates increasing prevalence and changes in disease stage presentation over time.

    Article  Google Scholar 

  13. Ito T, Sasano H, Tanaka M, Osamura R, Sasaki I, Kimura W, et al. Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan. J Gastroenterol. 2010;45(2):234–43.

    Article  Google Scholar 

  14. Zhang X, Ma L, Bao H, Zhang J, Wang Z, Gong P. Clinical, pathological and prognostic characteristics of gastroenteropancreatic neuroendocrine neoplasms in China: a retrospective study. BMC Endocr Disord. 2014;14:54.

    Article  Google Scholar 

  15. Fan JH, Zhang YQ, Shi SS, Chen Y, Yuan X, Jiang L, et al. A nation-wide retrospective epidemiological study of gastroenteropancreatic neuroendocrine neoplasms in china. Oncotarget. 2017;8(42):71699–708.

    Article  Google Scholar 

  16. Cho MY, Kim J, Sohn J, Kim M, Kim K, Kim W, et al. Current trends of the incidence and pathological diagnosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in Korea 2000-2009: multicenter study. Cancer Res Treat. 2012;44(3):157–65.

    Article  Google Scholar 

  17. Lim C, Lee I, Jun B, Kim J, Cho Y, Park J, et al. Incidence and clinical characteristics of gastroenteropancreatic neuroendocrine tumor in Korea: a single-center experience. Korean J Intern Med. 2017;32(3):452–8.

    Article  Google Scholar 

  18. Tsai HJ, Wu CC, Tsai CR, Lin S, Chen L, Chang J. The epidemiology of neuroendocrine tumors in Taiwan: a nation-wide cancer registry-based study. PLoS One. 2013;8(4):e62487.

    Article  CAS  Google Scholar 

  19. Sandvik O, Soreide K, Gudlaugsson E, Kvaloy J, Soreide J. Epidemiology and classification of gastroenteropancreatic neuroendocrine neoplasms using current coding criteria. Br J Surg. 2016;103(3):226–32.

    Article  CAS  Google Scholar 

  20. Hauso O, Gustafsson B, Kidd M, Waldum H, Drozdov I, Chan A, et al. Neuroendocrine tumor epidemiology. Cancer. 2008;113(10):2655–64.

    Article  Google Scholar 

  21. Gudmundsdottir H, Moller P, Jonasson J, Bjornsson E. Gastroenteropancreatic neuroendocrine tumors in Iceland: a population-based study. Scand J Gastroenterol. 2019;54(1):69–75 An analysis which demonstrated Iceland to be the rare country in which GEP NET incidence has not increased over the last several decades.

    Article  Google Scholar 

  22. Scherubl H, Streller B, Stabenow R, Herbst H, Hopfner M, Schwertner C, et al. Clinically detected gastroenteropancreatic neuroendocrine tumors are on the rise: epidemiological changes in Germany. World J Gastroenterol. 2013;19(47):9012–9.

    Article  Google Scholar 

  23. Ellis L, Shale M, Coleman M. Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenterol. 2010;105(12):2563–9.

    Article  Google Scholar 

  24. Genus T, Bouvier C, Wong K, Shirajaskanthan R, Rous B, Talbot D, et al. Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013-2015. Br J Cancer. 2019;121(11):966–72.

    Article  Google Scholar 

  25. Garcia-Carbonero R, Capdevila J, Crespo-Herrero G, Diaz-Perez J, Del Prado M, Orduna V, et al. 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. 2010;21(9):1794–803.

    Article  CAS  Google Scholar 

  26. Nikou G, Pazaitou-Panayiotou K, Dimitroulopoulos D, Alexandrakis A, Papakostas P, Vaslamatzis M, et al. Results of a prospective multicenter neuroendocrine tumor registry reporting on clinicopathologic characteristics of Greek patients. BMC Endocr Disord. 2016;16:8.

    Article  Google Scholar 

  27. Santos AP, Vinagre J, Soares P, Claro I, Sanches A, Gomes L, et al. Gastroenteropancreatic neuroendocrine neoplasia characterization in Portugal: results from the NETs Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism. Int J Endocrinol. 2019;2019:4518742.

    Article  CAS  Google Scholar 

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Correspondence to Satya Das.

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Conflict of Interest

Satya Das has received research funding from Exelixis and Entrinsic Health, and received compensation from Ipsen for previously serving on a speaker's bureau. Arvind Dasari has received research funding from Novartis / Advanced Accelerator Applications (AAA), Hutchison MediPharma, and Eisai, and has served on advisory boards for Ipsen, Novartis / AAA, Lexicon, and Crinetics.

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This article is part of the Topical Collection on Neuroendocrine Neoplasms

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Das, S., Dasari, A. Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?. Curr Oncol Rep 23, 43 (2021). https://doi.org/10.1007/s11912-021-01029-7

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