Pancreatic neuroendocrine tumors: Challenges in an underestimated disease

Crit Rev Oncol Hematol. 2016 May:101:193-206. doi: 10.1016/j.critrevonc.2016.03.013. Epub 2016 Mar 11.

Abstract

Pancreatic neuroendocrine tumours (PanNETs) are considered a relatively unusual oncologic entity. Due to its relative good prognosis, surgery remains the goal standard therapy not only in localized disease but also in the setting of locally or metastatic disease. Most of the patients are diagnosed in metastatic scenario, where multidisciplinary approach based on surgery, chemotherapies, liver-directed and/or molecular targeted therapies are commonly used. Owing to a deeper molecular knowledge of this disease, these targeted therapies are nowadays widely implemented, being the likely discovery of predictive biomarkers that would allow its use in other settings. This review is focused on describing the different classifications, etiology, prognostic biomarkers and multidisciplinary approaches that are typically used in PanNET.

Keywords: Foregut; Gastrointestinal neuroendocrine tumors; Pancreas surgery; Pancreatic Neuroendocrine Tumor (PanNET); Targeted therapies.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / blood
  • Humans
  • Molecular Targeted Therapy
  • Neoplasm Staging
  • Neuroendocrine Tumors* / diagnosis
  • Neuroendocrine Tumors* / drug therapy
  • Pancreatic Neoplasms* / drug therapy
  • Pancreatic Neoplasms* / pathology
  • Prognosis

Substances

  • Biomarkers, Tumor