Published online Apr 15, 2016. doi: 10.4251/wjgo.v8.i4.389
Peer-review started: May 25, 2015
First decision: September 14, 2015
Revised: January 18, 2016
Accepted: February 14, 2016
Article in press: February 16, 2016
Published online: April 15, 2016
Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs) represents a various family of rare tumours. Surgery is the first choice in GEP-NENs patients with localized disease whilst in the metastatic setting many other treatment options are available. Somatostatin analogues are indicated for symptoms control in functioning tumours. Furthermore they may be effective to inhibit tumour progression. GEP-NENs pathogenesis has been extensively studied in the last years therefore several driver mutations pathway genes have been identified as crucial factors in their tumourigenesis. GEP-NENs can over-express vascular endothelial growth factor (VEGF), basic-fibroblastic growth factor, transforming growth factor (TGF-α and -β), platelet derived growth factor (PDGF), insulin-like growth factor-1 (IGF-1) and their receptors PDGF receptor, IGF-1 receptor, epidermal growth factor receptor, VEGF receptor, and c-kit (stem cell factor receptor) that can be considered as potential targets. The availability of new targeted agents, such as everolimus and sunitinib that are effective in advanced and metastatic pancreatic neuroendocrine tumours, has provided new treatment opportunities. Many trials combing new drugs are ongoing.
Core tip: In this review, recent evidences in the biology and pathology of neuroendocrine neoplasms of the gastro-entero-pancreatic system were analysed, focusing on new biological perspectives of medical treatment. The evidence-based data of new-targeted drugs and the new molecular knowledge are summarized looking at the basis for future studies.