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Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans

The molecular pathogenesis of dermatofibrosarcoma protuberans (DFSP) involves distinctive rearrangement of chromosomes 17 and 22 leading to formation of the COL1A1-PDGFB fusion gene. The knowledge of molecular events underlying development of DFSP resulted in the implementation of targeted therapy w...

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Detalles Bibliográficos
Autores principales: Rutkowski, Piotr, Wozniak, Agnieszka, Switaj, Tomasz
Formato: Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3087969/
https://www.ncbi.nlm.nih.gov/pubmed/21559214
http://dx.doi.org/10.1155/2011/959132
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author Rutkowski, Piotr
Wozniak, Agnieszka
Switaj, Tomasz
author_facet Rutkowski, Piotr
Wozniak, Agnieszka
Switaj, Tomasz
author_sort Rutkowski, Piotr
collection PubMed
description The molecular pathogenesis of dermatofibrosarcoma protuberans (DFSP) involves distinctive rearrangement of chromosomes 17 and 22 leading to formation of the COL1A1-PDGFB fusion gene. The knowledge of molecular events underlying development of DFSP resulted in the implementation of targeted therapy with imatinib—a tyrosine kinase inhibitor (TKI), to the clinical practice. The striking efficacy of imatinib in advanced cases of DFSP has been demonstrated in a few clinical trials. Thus, imatinib is currently considered the gold standard in the treatment of inoperable and/or metastatic and/or recurrent cases of DFSP. Therapy with imatinib may potentially facilitate resection or decrease possible disfigurement related to radical surgical procedure. Following partial response on imatinib significant percentage of patients may be rendered free of the disease by surgery of the residual tumor.
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spelling pubmed-30879692011-05-10 Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans Rutkowski, Piotr Wozniak, Agnieszka Switaj, Tomasz Sarcoma Review Article The molecular pathogenesis of dermatofibrosarcoma protuberans (DFSP) involves distinctive rearrangement of chromosomes 17 and 22 leading to formation of the COL1A1-PDGFB fusion gene. The knowledge of molecular events underlying development of DFSP resulted in the implementation of targeted therapy with imatinib—a tyrosine kinase inhibitor (TKI), to the clinical practice. The striking efficacy of imatinib in advanced cases of DFSP has been demonstrated in a few clinical trials. Thus, imatinib is currently considered the gold standard in the treatment of inoperable and/or metastatic and/or recurrent cases of DFSP. Therapy with imatinib may potentially facilitate resection or decrease possible disfigurement related to radical surgical procedure. Following partial response on imatinib significant percentage of patients may be rendered free of the disease by surgery of the residual tumor. Hindawi Publishing Corporation 2011 2011-03-30 /pmc/articles/PMC3087969/ /pubmed/21559214 http://dx.doi.org/10.1155/2011/959132 Text en Copyright © 2011 Piotr Rutkowski et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Rutkowski, Piotr
Wozniak, Agnieszka
Switaj, Tomasz
Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans
title Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans
title_full Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans
title_fullStr Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans
title_full_unstemmed Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans
title_short Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans
title_sort advances in molecular characterization and targeted therapy in dermatofibrosarcoma protuberans
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3087969/
https://www.ncbi.nlm.nih.gov/pubmed/21559214
http://dx.doi.org/10.1155/2011/959132
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