Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 May 20;23(7):85.
doi: 10.1007/s11912-021-01074-2.

How to Manage Small Intestine (Jejunal and Ileal) Neuroendocrine Neoplasms Presenting with Liver Metastases?

Bruno Niederle  1 , Andreas Selberherr  2 , Martin B Niederle  3
Affiliations
Review

How to Manage Small Intestine (Jejunal and Ileal) Neuroendocrine Neoplasms Presenting with Liver Metastases?

Bruno Niederle et al. Curr Oncol Rep. .

Abstract

Purpose of review: Small intestinal neuroendocrine neoplasms (siNENs) are slowly growing tumours with a low malignant potential. However, more than half of the patients present with distant metastases (stage IV) and nearly all with locoregional lymph node (LN) metastases at the time of surgery. The value of locoregional treatment is discussed controversially.

Recent findings: In stage I to III disease, locoregional surgery was currently shown to be curative prolonging survival. In stage IV disease, surgery may prolong survival in selected patients with the chance to cure locoregional disease besides radical/debulking liver surgery. It may improve the quality of life and may prevent severe local complications resulting in a state of chronic malnutrition and severe intestinal ischaemia or bowel obstruction. Locoregional tumour resection offers the opportunity to be curative or to focus therapeutically on liver metastasis, facilitating various other therapeutic modalities. Risks and benefits of the surgical intervention need to be balanced individually.

Keywords: Neuroendocrine tumour (neoplasm), Small bowel, Jejunum, Ileum, Locoregional surgery, Stage IV.

PubMed Disclaimer

Conflict of interest statement

Bruno Niederle, Andreas Selberherr, and Martin B. Niederle declare that they have no conflict of interest.

References

    1. Niederle MB, Hackl M, Kaserer K, Niederle B. 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. 2010;17(4):909–918. doi: 10.1677/ERC-10-0152. - DOI - PubMed
    1. Bilimoria KY, Bentrem DJ, Wayne JD, Ko CY, Bennett CL, Talamonti MS. Small bowel cancer in the United States: changes in epidemiology, treatment, and survival over the last 20 years. Ann Surg. 2009;249(1):63–71. doi: 10.1097/SLA.0b013e31818e4641. - DOI - PubMed
    1. Kloppel G, Rindi G, Anlauf M, Perren A, Komminoth P. Site-specific biology and pathology of gastroenteropancreatic neuroendocrine tumors. Virchows Arch. 2007;451(Suppl 1):S9–27. doi: 10.1007/s00428-007-0461-0. - DOI - PubMed
    1. Sun Y, Lohse C, Smyrk T, Hobday T, Kroneman T, Zhang L. The influence of tumor stage on the prognostic value of Ki-67 index and mitotic count in small intestinal neuroendocrine tumors. Am J Surg Pathol. 2018;42(2):247–255. doi: 10.1097/PAS.0000000000000968. - DOI - PubMed
    1. Norlen O, Stalberg P, Oberg K, Eriksson J, Hedberg J, Hessman O, et al. Long-term results of surgery for small intestinal neuroendocrine tumors at a tertiary referral center. World J Surg. 2012;36(6):1419–1431. doi: 10.1007/s00268-011-1296-z. - DOI - PubMed

Publication types