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Genomic Profiles and Current Therapeutic Agents in Neuroendocrine Neoplasms.

Authors
  • Ohmoto, Akihiro1
  • Morizane, Chigusa2
  • 1 Division of Medical Oncology, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo 1358550, Japan. , (Japan)
  • 2 Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo 1040045, Japan. , (Japan)
Type
Published Article
Journal
Current drug targets
Publication Date
Jan 01, 2020
Volume
21
Issue
4
Pages
389–405
Identifiers
DOI: 10.2174/1389450119666191014105211
PMID: 31633473
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Neuroendocrine neoplasms (NENs) are rare tumors that mainly occur in the gastroenteropancreatic (GEP) tract and lungs. According to the current World Health Organization classification for GEP-NENs and lung NENs, treatment strategies differ for well-differentiated and poorly differentiated subtypes. For well-differentiated GEP-NENs, somatostatin analogues (SSA), peptide receptor radionuclide therapy, and molecular-targeted agents are approved as the standards of care based on phase III clinical trial data. Promising data regarding the use of everolimus and the novel SSA pasireotide for lung NENs are emerging, though additional studies are required to confirm these effects. For poorly differentiated tumors from the GEP tract and lung, a platinum-based cytotoxic regimen is widely used. Genomic analysis has recently revealed a diverse pattern of primary organ-dependent mutations, and the use of traditional treatment strategies versus organ-specific strategies is currently under discussion. In addition, clinical trials for several molecular-targeted agents and immune checkpoint inhibitors for the treatment of NENs are currently underway. Accumulating genomic information is expected to contribute to the development of novel therapies for other organ-derived NENs or poorly differentiated neuroendocrine carcinomas (NECs). Here, we provide an updated overview of the current knowledge regarding genomic profiles and representative agents for NENs and highlight the prospects for future investigations. Copyright© Bentham Science Publishers; For any queries, please email at [email protected]

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