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Molecular prognostic factors in small-intestinal neuroendocrine tumours
BACKGROUND: Small-intestinal neuroendocrine tumours (SI-NETs) represent a heterogeneous group of rare tumours. In recent years, basic research in SI-NETs has attempted to unravel the molecular events underlying SI-NET tumorigenesis. AIM: We aim to provide an overview of the current literature regard...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Bioscientifica Ltd
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6599083/ https://www.ncbi.nlm.nih.gov/pubmed/31189127 http://dx.doi.org/10.1530/EC-19-0206 |
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author | Samsom, K G van Veenendaal, L M Valk, G D Vriens, M R Tesselaar, M E T van den Berg, J G |
author_facet | Samsom, K G van Veenendaal, L M Valk, G D Vriens, M R Tesselaar, M E T van den Berg, J G |
author_sort | Samsom, K G |
collection | PubMed |
description | BACKGROUND: Small-intestinal neuroendocrine tumours (SI-NETs) represent a heterogeneous group of rare tumours. In recent years, basic research in SI-NETs has attempted to unravel the molecular events underlying SI-NET tumorigenesis. AIM: We aim to provide an overview of the current literature regarding prognostic and predictive molecular factors in patients with SI-NETs. METHOD: A PubMed search was conducted on (epi)genetic prognostic factors in SI-NETs from 2000 until 2019. RESULTS: The search yielded 1522 articles of which 20 reviews and 35 original studies were selected for further evaluation. SI-NETs are mutationally quiet tumours with a different genetic make-up compared to pancreatic NETs. Loss of heterozygosity at chromosome 18 is the most frequent genomic aberration (44–100%) followed by mutations of CDKN1B in 8%. Prognostic analyses were performed in 16 studies, of which 8 found a significant (epi)genetic association for survival or progression. Loss of heterozygosity at chromosome 18, gains of chromosome 4, 5, 7, 14 and 20p, copy gain of the SRC gene and low expression of RASSF1A and P16 were associated with poorer survival. In comparison with genetic mutations, epigenetic alterations are significantly more common in SI-NETs and may represent more promising targets in the treatment of SI-NETs. CONCLUSION: SI-NETs are mutationally silent tumours. No biomarkers have been identified yet that can easily be adopted into current clinical decision making. SI-NETs may represent a heterogeneous disease and larger international studies are warranted to translate molecular findings into precision oncology. |
format | Online Article Text |
id | pubmed-6599083 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Bioscientifica Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-65990832019-07-03 Molecular prognostic factors in small-intestinal neuroendocrine tumours Samsom, K G van Veenendaal, L M Valk, G D Vriens, M R Tesselaar, M E T van den Berg, J G Endocr Connect Research BACKGROUND: Small-intestinal neuroendocrine tumours (SI-NETs) represent a heterogeneous group of rare tumours. In recent years, basic research in SI-NETs has attempted to unravel the molecular events underlying SI-NET tumorigenesis. AIM: We aim to provide an overview of the current literature regarding prognostic and predictive molecular factors in patients with SI-NETs. METHOD: A PubMed search was conducted on (epi)genetic prognostic factors in SI-NETs from 2000 until 2019. RESULTS: The search yielded 1522 articles of which 20 reviews and 35 original studies were selected for further evaluation. SI-NETs are mutationally quiet tumours with a different genetic make-up compared to pancreatic NETs. Loss of heterozygosity at chromosome 18 is the most frequent genomic aberration (44–100%) followed by mutations of CDKN1B in 8%. Prognostic analyses were performed in 16 studies, of which 8 found a significant (epi)genetic association for survival or progression. Loss of heterozygosity at chromosome 18, gains of chromosome 4, 5, 7, 14 and 20p, copy gain of the SRC gene and low expression of RASSF1A and P16 were associated with poorer survival. In comparison with genetic mutations, epigenetic alterations are significantly more common in SI-NETs and may represent more promising targets in the treatment of SI-NETs. CONCLUSION: SI-NETs are mutationally silent tumours. No biomarkers have been identified yet that can easily be adopted into current clinical decision making. SI-NETs may represent a heterogeneous disease and larger international studies are warranted to translate molecular findings into precision oncology. Bioscientifica Ltd 2019-06-10 /pmc/articles/PMC6599083/ /pubmed/31189127 http://dx.doi.org/10.1530/EC-19-0206 Text en © 2019 The authors http://creativecommons.org/licenses/by-nc/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. (http://creativecommons.org/licenses/by-nc/4.0/) |
spellingShingle | Research Samsom, K G van Veenendaal, L M Valk, G D Vriens, M R Tesselaar, M E T van den Berg, J G Molecular prognostic factors in small-intestinal neuroendocrine tumours |
title | Molecular prognostic factors in small-intestinal neuroendocrine tumours |
title_full | Molecular prognostic factors in small-intestinal neuroendocrine tumours |
title_fullStr | Molecular prognostic factors in small-intestinal neuroendocrine tumours |
title_full_unstemmed | Molecular prognostic factors in small-intestinal neuroendocrine tumours |
title_short | Molecular prognostic factors in small-intestinal neuroendocrine tumours |
title_sort | molecular prognostic factors in small-intestinal neuroendocrine tumours |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6599083/ https://www.ncbi.nlm.nih.gov/pubmed/31189127 http://dx.doi.org/10.1530/EC-19-0206 |
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