4.7 Review

Molecular Signatures and Their Clinical Utility in Pancreatic Neuroendocrine Tumors

Journal

FRONTIERS IN ENDOCRINOLOGY
Volume 11, Issue -, Pages -

Publisher

FRONTIERS MEDIA SA
DOI: 10.3389/fendo.2020.575620

Keywords

molecular signatures; signaling pathway; tumorigenesis; clinical trial; neuroendocrine carcinoma; pancreatic neuroendocrine tumor

Funding

  1. Intramural Research Program of the National Institutes of Health: NCI
  2. Intramural Research Program of the National Institutes of Health: NIDDK
  3. ZIA [DK075035-12]

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PNETs are classified based on histologic differentiation and proliferative indices, with advances in high-throughput techniques allowing researchers to explore genetic alterations associated with malignant transformation. Clinical aggressiveness stratifies along other genotypic and phenotypic demarcations, impacting the potential molecular signatures, associated trials, and differences in responses to therapies for PNETs.
Pancreatic neuroendocrine tumors (PNETs) are classified based on their histologic differentiation and proliferative indices, which have been used extensively to determine prognosis. Advances in next-generation sequencing and other high-throughput techniques have allowed researchers to objectively explore tumor specimens and learn about the genetic alterations associated with malignant transformation in PNETs. As a result, targeted, pathway-specific therapies have been emerging for the treatment of unresectable and metastatic disease. As we continue to trial various pharmaceutical products, evidence from studies using multi-omics approaches indicates that clinical aggressiveness stratifies along other genotypic and phenotypic demarcations, as well. In this review, we explore the clinically relevant and potentially targetable molecular signatures of PNETs, their associated trials, and the overall differences in reported prognoses and responses to existing therapies.

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