Molecular prognostic factors in small-intestinal neuroendocrine tumours

K G Samsom1, L M van Veenendaal2, G D Valk3

  • 1Department of Pathology, Netherlands Cancer Institute, Amsterdam, The Netherlands.

Endocrine Connections
|June 13, 2019
PubMed
Abstract

Insights

Small-intestinal neuroendocrine tumours (SI-NETs) are genetically quiet. Epigenetic alterations, not genetic mutations, are more common and may offer better therapeutic targets for these rare tumors.

Area of Science:

  • Oncology
  • Genetics
  • Molecular Biology

Background:

  • Small-intestinal neuroendocrine tumours (SI-NETs) are rare and heterogeneous.
  • Understanding the molecular basis of SI-NET tumorigenesis is crucial.

Purpose of the Study:

  • To review prognostic and predictive molecular factors in SI-NETs.
  • To identify key genetic and epigenetic alterations associated with SI-NET outcomes.

Main Methods:

  • Conducted a PubMed literature search for (epi)genetic prognostic factors in SI-NETs (2000-2019).
  • Evaluated 20 reviews and 35 original studies from 1522 initial articles.

Main Results:

  • SI-NETs exhibit distinct genetic profiles compared to pancreatic NETs, with loss of heterozygosity at chromosome 18 being frequent.
  • Significant associations between specific genomic aberrations (e.g., chromosome 18 LOH, chromosome 4, 5, 7, 14, 20p gains) and poorer survival were identified.
  • Epigenetic alterations are more prevalent than genetic mutations in SI-NETs and show promise as therapeutic targets.

Conclusions:

  • SI-NETs are characterized by a low mutational burden.
  • Currently, no specific biomarkers are readily applicable for clinical decision-making in SI-NETs.
  • Further large-scale international studies are needed to translate molecular findings into precision oncology for this heterogeneous disease.

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