Skip to main content
Log in

Surgical Management of Small Bowel Neuroendocrine Tumors: Specific Requirements and Their Impact on Staging and Prognosis

  • Endocrine Tumors
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Small bowel neuroendocrine tumors (SB-NETs) are characterized by two main features: they usually are metastatic at diagnosis and multiple in 30 % of cases. As such, SB-NETs require specific surgical management. This retrospective study examined local recurrence, survival, and prognosis of SB-NETs after adapted surgery.

Methods

All consecutive patients with SB-NETs who underwent resection of at least one primary tumor between 1 January 2000 and 1 January 2013 were analyzed. The preoperative morphologic workup, histologic classification, and metastatic lymph node (LN) ratio (LNs involved/removed) were recorded.

Results

The study enrolled 107 patients, 35 (33 %) of whom had multiple SB-NETs (range 1–44; mean 3.1). Preoperative imaging and perioperative surgical examination missed 61 and 33 % of SB-NETs, respectively, in contrast to pathologic examination. Of the 107 patients, 43 % had carcinoid syndrome, 70 % had metastatic disease, and 90 % had LN involvement. The median number of LNs retrieved was 12 (range 1–69). The LN ratio (LNs involved/removed) was 0.25. The highest tumoral grades were G1 (in 61 % of patients) and G2 (in 37 % of patients). Of the 107 patients, 13 (12 %) had local LN recurrence. The rate of LN recurrence-free survival at 5 years was 88 %. The median overall survival (OS) time was 128 months (range 91–165 months). In the multivariate analysis, high chromogranin A (CgA) levels and peritoneal carcinomatosis were significantly associated with shorter OS.

Conclusions

Systematic palpation of the entire small bowel detects more multiple NETs than preoperative imaging. Systematic surgery with extensive LN resection is associated with low local recurrence. High CgA levels and carcinomatosis are linked with shorter survival.

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
Fig. 2

Similar content being viewed by others

References

  1. Yao JC, Hassan M, Phan A, 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;26:3063–72.

    Article  PubMed  Google Scholar 

  2. Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9:61–72.

    Article  PubMed  CAS  Google Scholar 

  3. Ferrone CR. Lymphadenectomy for pancreatic neuroendocrine tumors: is that the relevant debate? Ann Surg. 2014;259:213–4.

    Article  PubMed  Google Scholar 

  4. Eriksson B, Kloppel G, Krenning E, et al. Consensus guidelines for the management of patients with digestive neuroendocrine tumors: well-differentiated jejunal-ileal tumor/carcinoma. Neuroendocrinology. 2008;87:8–19.

    Article  PubMed  CAS  Google Scholar 

  5. Boudreaux JP, Klimstra DS, Hassan MM, et al. The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the jejunum, ileum, appendix, and cecum. Pancreas. 2010;39:753–66.

    Article  PubMed  Google Scholar 

  6. Ramage JK, Ahmed A, Ardill J, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2011;61:6–32.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Hellman P, Lundstrom T, Ohrvall U, et al. Effect of surgery on the outcome of midgut carcinoid disease with lymph node and liver metastases. World J Surg. 2002;26:991–7.

    Article  PubMed  Google Scholar 

  8. Givi B, Pommier SJ, Thompson AK, et al. Operative resection of primary carcinoid neoplasms in patients with liver metastases yields significantly better survival. Surgery. 2006;140:891–7; discussion 897–8.

    Article  PubMed  Google Scholar 

  9. Strosberg J, Gardner N, Kvols L. Survival and prognostic factor analysis of 146 metastatic neuroendocrine tumors of the mid-gut. Neuroendocrinology. 2009;89:471–6.

    Article  PubMed  CAS  Google Scholar 

  10. Capurso G, Rinzivillo M, Bettini R, et al. Systematic review of resection of primary midgut carcinoid tumour in patients with unresectable liver metastases. Br J Surg. 2012;99:1480–6.

    Article  PubMed  CAS  Google Scholar 

  11. van Tuyl SA, van Noorden JT, Timmer R, et al. Detection of small-bowel neuroendocrine tumors by video capsule endoscopy. Gastrointest Endosc. 2006;64:66–72.

    Article  PubMed  Google Scholar 

  12. Kamaoui I, De-Luca V, Ficarelli S, et al. Value of CT enteroclysis in suspected small-bowel carcinoid tumors. AJR Am J Roentgenol. 2010;194:629–33.

    Article  PubMed  Google Scholar 

  13. Landry CS, Lin HY, Phan A, et al. Resection of at-risk mesenteric lymph nodes is associated with improved survival in patients with small bowel neuroendocrine tumors. World J Surg. 2013;37:1695–700.

    Article  PubMed  Google Scholar 

  14. Wang YZ, Joseph S, Lindholm E, et al. Lymphatic mapping helps to define resection margins for midgut carcinoids. Surgery. 2009;146:993–7.

    Article  PubMed  Google Scholar 

  15. Strosberg JR, Cheema A, Weber J, et al. Prognostic validity of a novel American Joint Committee on Cancer Staging Classification for pancreatic neuroendocrine tumors. J Clin Oncol. 2013;29:3044–9.

    Article  Google Scholar 

  16. Durante C, Boukheris H, Dromain C, et al. Prognostic factors influencing survival from metastatic (stage IV) gastroenteropancreatic well-differentiated endocrine carcinoma. Endocr Relat Cancer. 2009;16:585–97.

    Article  PubMed  Google Scholar 

  17. Rindi G, Kloppel G, Couvelard A, et al. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2007;451:757–62.

    Article  PubMed  CAS  Google Scholar 

  18. Bosman FT CF, Hruban RH. WHO classification of tumours of the digestive system. IARC, Lyon, 2010.

    Google Scholar 

  19. Sobin LH, Gospodarowicz M, Wittekind C (2009) TNM classification of malignant tumours. 7th ed. Wiley-Blackwell, Hoboken.

    Google Scholar 

  20. Norlen O, Stalberg P, Oberg K, et al. Long-term results of surgery for small intestinal neuroendocrine tumors at a tertiary referral center. World J Surg. 2012;36:1419–31.

    Article  PubMed  Google Scholar 

  21. Landerholm K, Zar N, Andersson RE, et al. Survival and prognostic factors in patients with small bowel carcinoid tumour. Br J Surg. 2011;98:1617–24.

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interest

There are no conflicts of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Arnaud Pasquer MD.

Appendix

Appendix

See Appendix Tables 4 and 5.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pasquer, A., Walter, T., Hervieu, V. et al. Surgical Management of Small Bowel Neuroendocrine Tumors: Specific Requirements and Their Impact on Staging and Prognosis. Ann Surg Oncol 22 (Suppl 3), 742–749 (2015). https://doi.org/10.1245/s10434-015-4620-2

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1245/s10434-015-4620-2

Keywords

Navigation