Skip to main content

Advertisement

Log in

Management of Locally Advanced and Unresectable Small Bowel Neuroendocrine Tumours

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

Abstract

Three subtypes of small bowel neuroendocrine tumours (SBNETs) have been described: Type A: SBNET with resectable mesenteric disease that does not involve the mesenteric root; Type B: “Borderline resectable” SBNET presenting with mesenteric nodal metastases and fibrosis adjacent but not encasing the main trunk of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV); and Type C: “Locally advanced or irresectable” SBNET where tumour deposits and fibrosis encase the SMA and SMV. Type C SBNETs are rare and constitute around 5% of patients in reported series, although this may underestimate the prevalence. In these patients, almost all will present with symptoms of intestinal ischemia or obstruction and symptom management should be a primary main focus of treatment. All patients should be carefully staged with cross-sectional imaging and 68 Ga-dotate positron emission tomography, and discussed at a dedicated neuroendocrine tumour multidisciplinary meeting. Expert surgical review should always be sought as experienced centers have a high rate of successful resection of primary tumours and mesenteric disease. If resection is not feasible, surgical bypass should be considered in patients with a discrete and symptomatic point of obstruction. Non-operative management should emphasize symptomatic treatment with somatostatin analogs, nutritional advice and support and palliative care. Successful neoadjuvant approaches utilizing peptide radionucleide receptor therapy and systemic chemotherapy with everolimus or temazolamide/capecitabine have not been reported.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Dasari A, Shen C, Halperin D et al (2017) Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol 3:1335–1342

    Article  Google Scholar 

  2. Chan DL, Dixon M, Law CL et al (2018) Outcomes of cytoreductive surgery for metastatic low-grade neuroendocrine tumors in the setting of extrahepatic metastases. Ann Surg Oncol 25(6):1768–1774

    Article  Google Scholar 

  3. Erikson J, Garmo H, Hellman P et al (2017) The influence of preoperative symptoms on the death of patients with small intestinal neuroendocrine tumours. Ann Surg Oncol 24:1214–1220

    Article  Google Scholar 

  4. Ellis L, Shale MJ, Coleman MP (2010) Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenterol 105:2563–2569

    Article  Google Scholar 

  5. Niederle B, Pape UF, Costa F et al (2016) ENETS consensus guidelines update for neuroendocrine neoplasm of the jejunum and ileum. Neuroendocrinology 103(2):125–138

    Article  CAS  Google Scholar 

  6. Laskaratos FM, Diamantopoulos L, Walker M et al (2018) Prognostic factors for survival among patients with small bowel neuroendocrine tumours associated with mesenteric desmoplasia. Neuroendocrinology 1:1–15. https://doi.org/10.1159/00486097

    Article  Google Scholar 

  7. Chambers AJ, Pasieka JL, Dixon E et al (2008) The palliative benefit of aggressive surgical intervention for both hepatic and mesenteric metastases from neuroendocrine tumors. Surgery 144:645–651 Discussion 651-653

    Article  Google Scholar 

  8. Pasquer A, Walter T, Hervieu V et al (2015) Surgical management of small bowel neuroendocrine tumors: specific requirements and their impact on staging and prognosis. Ann Surg Oncol 22(Suppl 3):S742–S749

    Article  Google Scholar 

  9. Watzka FM, Fottner C, Miederer M et al (2016) Surgical treatment of NEN of small bowel: a retrospective analysis. World J Surg 40:749–758. https://doi.org/10.1007/s00268-016-3432-2

    Article  CAS  PubMed  Google Scholar 

  10. Gaujoux S, Sauvanet A, Belghiti J (2012) Place of surgical resection in the treatment strategy for gastrointestinal neuroendocrine tumors. Targ Oncol 7:153–159

    Article  Google Scholar 

  11. Blazevic A, Zandee WT, Franssen GJH et al (2018) Mesenteric fibrosis and palliative surgery in small intestinal neuroendocrine tumours. Endocr Relat Cancer 25:245–254

    Article  CAS  Google Scholar 

  12. de Mestier L, Lardiere-Deguelte S, Brixi H et al (2015) Updating the surgical management of peritoneal carcinomatosis in patients with neuroendocrine tumors. Neuroendocrinology 101:105–111

    Article  Google Scholar 

  13. Manguso N, Gangi A, Nissen N et al (2018) Long-term outcomes after elective versus emergency surgery for small bowel endocrine tumors. Am Surg 84:1570–1574

    Article  Google Scholar 

  14. Partelli S, Bartsch DK, Capdevila J et al (2017) ENETS consensus guidelines for the standards of care in neuroendocrine tumours: surgery for small intestinal and pancreatic neuroendocrine tumours. Neuroendocrinology 105:255–265

    Article  CAS  Google Scholar 

  15. Pantongrag-Brown L, Buetow PC, Carr NJ et al (1995) Calcification and fibrosis in mesenteric carcinoid tumor: CT findings and pathologic correlation. Am J Radiol 164:387–391

    CAS  Google Scholar 

  16. Laskaratos F-M, Rombouts K, Caplin M et al (2017) Neuroendocrine tumours and fibrosis: an unsolved mystery. Cancer 123:4770–4790

    Article  Google Scholar 

  17. Cirri P, Cihiarugi P (2012) Cancer-associated-fibroblasts and tumour cells: a diabolic liason driving cancer progression. Cancer Metastasis Rev 31:195–208

    Article  Google Scholar 

  18. Nebigil CG, Launay LM, Hickel P et al (2000) 5-hydroxytryptamine 2B receptor regulates cell-cycle progression: cross-talk with tyrosine kinase pathways. Proc Natl Acad Sci USA 97:2591–2596

    Article  CAS  Google Scholar 

  19. Launay JM, Birraux G, Bondoux D et al (1996) Ras involvement in signal transduction by the serotonin 5-HT2B receptor. J Biol Chem 271:3141–3147

    Article  CAS  Google Scholar 

  20. Woodard PK, Feldman JM, Paine SS et al (1995) Midgut carcinoid tumors: CT findings and biochemical profiles. J Comput Assist Tomogr 19:400–405

    Article  CAS  Google Scholar 

  21. Beauchamp RD, Coffey RJ Jr, Lyons RM et al (1991) Human carcinoid cell production of paracrine growth factors that can stimulate fibroblast and endothelial cell growth. Cancer Res 51:5253–5260

    CAS  PubMed  Google Scholar 

  22. Cunningham JL, Tsolakis AV, Jacobson A et al (2010) Connective tissue growth factor expression in endocrine tumors is associated with high stromal expression of alpha-smooth muscle actin. Eur J Endocrinol 163:691–697

    Article  CAS  Google Scholar 

  23. La Rosa S, Chiaravalli AM, Capella C et al (1997) Immunohistochemical localization of acidic fibroblast growth factor in normal human enterochromaffin cells and related gastrointestinal tumours. Virchows Arch 25:175–180

    Google Scholar 

  24. Pavel M, Valle JW, Eriksson B et al (2017) ENETS consensus guidelines for the standard of care in neuroendocrine neoplasms: Systemic therapy, biotherapy and novel targeted agents. Neuroendocrinology 105(3):266–280

    Article  CAS  Google Scholar 

  25. Lee L, Ito T, Jensen RT (2018) Everolimus in treatment of neuroendocrine tumors: efficacy, side effects, resistance and factors affecting its place in the treatment sequence. Expert Opin Pharmacother 19(8):909–928

    Article  CAS  Google Scholar 

  26. Ramirez RA, Beyer DT, Chauhan A et al (2016) The role of capecitabine/temozolomide in metastatic neuroendocrine tumors. Oncologist 21(6):671–675

    Article  Google Scholar 

  27. Hellman P, Hessman O, Akerstrom G, Stalberg P (2010) Stenting of the superior mesenteric vein in midgut carcinoid disease with large mesenteric masses. World J Surg 34(6):1373–1379. https://doi.org/10.1007/s00268-009-0361-3

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The CommNETS Surgical Section includes Dr Julie Hallett and Dr Calvin Law from the Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada; Dr Janice Pasieka, Tom Baker Cancer Centre, Alberta, Canada; Dr Jonathan Koea, North Shore Hospital, Auckland, New Zealand; Dr Win Meyer-Rochow, Waikato Hospital, Hamilton, New Zealand.

Funding

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

Authors

Consortia

Corresponding author

Correspondence to Jonathan Koea.

Additional information

Publisher's Note

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

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.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Koea, J., the Commonwealth Neuroendocrine Tumour Research Collaborative (CommNETs) Surgical Section. Management of Locally Advanced and Unresectable Small Bowel Neuroendocrine Tumours. World J Surg 45, 219–224 (2021). https://doi.org/10.1007/s00268-020-05740-7

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-020-05740-7

Navigation