Neuroendocrine tumors of the gastrointestinal tract and the role of cytotoxic chemotherapy

Expert Rev Anticancer Ther. 2016;16(4):391-401. doi: 10.1586/14737140.2016.1146135. Epub 2016 Feb 17.


Neuroendocrine tumors (NETs) comprise a heterogeneous group of tumors derived from peptide- and amine-secreting cells of the neuroendocrine system. NETs commonly arise in the gastrointestinal tract but can arise in most organs of the body. Although the incidence of NETs is not high, the prevalence is relatively high because many patients may live relatively long periods without major symptoms from the disease. Whilst many of these tumors take on an indolent clinical course, they constitute a therapeutic challenge when they progress, metastasize and become symptomatic. Treatment requires a multidisciplinary approach that may include cytotoxic chemotherapy. Almost all clinical trials investigating cytotoxic chemotherapy in NETs are small single-arm studies, hence guidelines are derived from expert opinion and from extrapolating results from small-cell lung cancer studies. This article provides a broad coverage of systemic therapies for NETs but focusses on the role of cytotoxic chemotherapy.

Keywords: cytotoxic chemotherapy; neuroendocrine carcinoma; neuroendocrine neoplasms; neuroendocrine tumors.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / pharmacology
  • Antineoplastic Agents / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Biomarkers, Tumor / analysis
  • Diagnostic Imaging / methods
  • Gastrointestinal Neoplasms / diagnostic imaging
  • Gastrointestinal Neoplasms / pathology*
  • Gastrointestinal Neoplasms / therapy*
  • Humans
  • Molecular Targeted Therapy
  • Neuroendocrine Tumors / diagnostic imaging
  • Neuroendocrine Tumors / pathology*
  • Neuroendocrine Tumors / therapy*
  • Radiotherapy / methods
  • Streptozocin / therapeutic use
  • Topotecan / therapeutic use


  • Antineoplastic Agents
  • Biomarkers, Tumor
  • Streptozocin
  • Topotecan