Skip to main content

Advertisement

SpringerLink
Log in

Multifocal Small Bowel Neuroendocrine Tumours

  • Surgical Symposium Contribution
  • Published:
World Journal of Surgery Aims and scope Submit manuscript

Abstract

In contemporary surgical series, the incidence of multifocal tumours (MFT) in small bowel neuroendrocrine tumours (SBNET) is as high as 50%. Familial forms of SBNET appear to have an even higher rate of MFTs in the range of 80%. Multifocal disease poses several challenges and questions for the operating surgeon, including the cause of the disease whether it is local metastases or a genetic field defect, the extent of the resection, and the prognosis. The etiology of these multiple tumours appears to be multifactorial and may represent localized metastases in some, a genetic mutation resulting in the rare familial form of this disease in others, or the integration of genetic and epigenetic events within the bowel. Contrary to historical series, recent data suggest that MFT are not associated with a poorer prognosis. The challenge remains on the long-term surveillance of these unique tumours.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Saleh MA, Mansoor E, Anindo M et al (2019) Prevalence of small intestine carcinoid tumors: a us population-based study 2012–2017. Dig Dis Sci 64:1328–1334

    Article  Google Scholar 

  2. Moertel CG, Sauer WG, Dokkert MB et al (1961) Life history of the carcinoid tumour of the small intestine. Cancer 14:901–912

    Article  CAS  Google Scholar 

  3. Yantiss RK, Odze RD, Farrave FA et al (2003) Solitary versus multiple carcinoid tumors of the ileum. Am J Surg Pathol 27:811–817

    Article  Google Scholar 

  4. Burke AP, Thomas RM, Elsayed AM et al (1997Carcinoids) Carcinoids of the jejunum and ileum: an immunohistochemical and clinicopathological study of 167 cases. Cancer 79:1086–1093

    Article  CAS  Google Scholar 

  5. Choi AB, Maxwell JE, Keck KJ et al (2017) Is multifocality an indicator of aggressive behavior in small bowel neuroendocrine tumors? Pancreas 46(9):1115–1120. https://doi.org/10.1097/MPA.0000000000000911

    Article  PubMed  PubMed Central  Google Scholar 

  6. Gangi A, Siegel E, Barmparas G et al (2018) multifocality in small bowel neuroendocrine tumors. J Gastrointest Surg 22:303–309

    Article  Google Scholar 

  7. Hughes MS, Azoury SC, Assadipour Y et al (2016) Prospective evaluation and treatment of familial carcinoid small intestine neuroendocrine tumors (SI-NETs). Surgery 159(1):350–356. https://doi.org/10.1016/j.surg.2015.05.041

    Article  PubMed  Google Scholar 

  8. Stalberg P, Westin G, Thirlwell C (2016) Genetics and epigenetics in small intestinal neuroendocrine tumours. J Intern Med 280(584):594

    Google Scholar 

  9. Sei Y, FengJ ZX et al (2016) Polyclonal crypt genesis and development of familial small intestinal neuroendocrine tumors. Gastroenterology 151(1):140–151. https://doi.org/10.1053/j.gastro.2016.03.007

    Article  PubMed  PubMed Central  Google Scholar 

  10. Wang YZ, Carrasquillo JP, McCord E et al (2014) Reappraisal of lymphatic mapping for midgut neuroendocrine patients undergoing cytoreductive surgery. Surgery 156:1498–1503

    Article  Google Scholar 

  11. Guo Z, Li Q, Wilander E, PonteÂn J et al (2000) Clonality analysis of multifocal carcinoid tumours of the small intestine by X-chromosome inactivation analysis. J Pathol 190:76–79

    Article  CAS  Google Scholar 

  12. Katona TM, Jones TD, Wang M et al (2006) Molecular evidence for independent origin of multifocal neuroendocrine tumors of the enteropancreatic axis. Cancer Res 66(9):4936–4942

    Article  CAS  Google Scholar 

  13. Berge T, Linell F (1976) Carcinoid tumours. frequency in a defined population during a 12 year period. Acta Pathol Microbiol Scand A 84:322–330

    CAS  PubMed  Google Scholar 

  14. Banck MS, Kanwar R, Kulkarni AA et al (2013) The genomic landscape of small intestine neuroendocrine tumors. J Clin Invest 123:2502–2505

    Article  CAS  Google Scholar 

  15. Crona J, Gustavsson T, Norlén O et al (2015) Somatic mutations and genetic heterogeneity at the CDKN1B locus in small intestinal neuroendocrine tumors. Ann Surg Oncol 3:1428–1435

    Article  Google Scholar 

  16. Francis JM, Kiezun A, Ramos AH et al (2013) Somatic mutation of CDKN1B in small intestine neuroendocrine tumors. Nat Genet 45(12):1483–1486. https://doi.org/10.1038/ng.2821

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Scarpa A (2019) The landscape of molecular alterations in pancreatic and small intestinal neuroendocrine tumours. Annales d’Endocrinologie 80:153–158

    Article  Google Scholar 

  18. Karpathakis A, Dibra H, Pipinikas C et al (2016) Prognostic impact of novel molecular subtypes of small intestinal neuroendocrine tumor. Clin Cancer Res 22(1):250–258

    Article  CAS  Google Scholar 

  19. Howe JR, Merchant NB, Conrad C et al (2020) The north american neuroendocrine tumor society consensus paper on the surgical management of pancreatic neuroendocrine tumors. Pancreas 49(1):1–33. https://doi.org/10.1097/MPA.0000000000001454

    Article  PubMed  Google Scholar 

  20. Zaidi MY, Lopez-Aguiar AG, Dillhoff M et al (2019) Prognostic role of lymph node positivity and number of lymph nodes needed for accurately staging small-bowel neuroendocrine tumors. JAMA Surg 154(2):134–140. https://doi.org/10.1001/jamasurg.2018.3865

    Article  PubMed  Google Scholar 

  21. Grandhi MS, Lafaro KJ, Pawlik TM (2015) Role of logo regional and systemic approaches for the treatment of patients with metastatic neuroendocrine tumors. J Gastrointest Surg 19(12):2273–2282

    Article  Google Scholar 

  22. Niederle B, Pape U, Costa F et al (2016) ENAETs consensus guidelines update for neuroendocrine neoplasms of the jejunum and ileum. Neuroendocrinology 103:125–138

    Article  CAS  Google Scholar 

  23. Kasai Y, Mahuron K, Hirose K et al (2019) Prognostic impact of a large mesenteric mass >2 cm in ileal neuroendocrine tumor. J Surg Oncol 120:1311–1317

    Article  Google Scholar 

  24. Landry CS, Lin HY, Phan A et al (2013) Resection of at risk mesenteric lymph nodes is associated with improved survival in patients with small bowel neuroendocrine tumors. World J Surg 37:1695–1700. https://doi.org/10.1007/s00268-013-1918-8

    Article  PubMed  Google Scholar 

  25. Bilimoria KY, Bentrem DJ, Wayne JD et al (2009) Small bowel cancer in USA; changes in epidemiology, treatment and survival over last 20 years. Ann Surg 249:63–71

    Article  Google Scholar 

  26. Ito T, Igarashi H, Nakamura K et al (2015) Epidemiological trends in pancreatic and gastrointestinal neuroendocrine tumors in Japan. J Gastroenterol 50:58–64

    Article  Google Scholar 

  27. Le Roux C, Lombard-Bohas C, Delmas C et al (2011) Relapse factors for ileal neuroendocrine tumours after curative surgery: a retrospective French multicentre study. Dig Liver Dis 43:828–833 [PubMed: 21641888]

    Article  Google Scholar 

  28. Singh S, Chan DL, Moody L et al (2018) 2Recurrence in resected gastroenteropancreatic neuroendocrine tumors. JAMA Oncol 4(4):583–585

    Article  Google Scholar 

  29. Singh S, Moody L, Chan DL et al (2018) Follow-up recommendations for completely resected gastroenteropancreatic neuroendocrine tumors. JAMA Oncol 4(11):1597–1604

    Article  Google Scholar 

  30. Strosberg JR, Halfdanarson TR, Bellizzi AM et al (2017) The north american neuroendocrine society (nanets) consensus guidelines for surveillance and medical management of midgut neuroendocrine tumors. Pancreas 46(6):707–714. https://doi.org/10.1097/MPA.0000000000000850

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

The CommNETS Collaboration is supported by unconditional educational grant from Ipsen Canada and an unconditional sponsorship grant from Ipsen Australia

Author information

Authors and Affiliations

  1. Department of Surgery, Sections General Surgery and Surgical Oncology, Cumming School of Medicine, University of Calgary, North Tower FMC, 1403 29th Street NW, Calgary, Alberta, T2N 2T9, Canada

    Janice L. Pasieka

Authors
  1. Janice L. Pasieka
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

This article is contribution to the Symposium entitled, “Surgical Dilemmas and Challenges for the Operating Surgeon in the Management of Small Bowel Neuroendocrine Tumours: A CommNETs Symposium”.

Corresponding author

Correspondence to Janice L. Pasieka.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pasieka, J.L. Multifocal Small Bowel Neuroendocrine Tumours. World J Surg 45, 208–212 (2021). https://doi.org/10.1007/s00268-020-05663-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-020-05663-3

Search

Navigation