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Thérapies ciblées et dermatofibrosarcome de Darier et Ferrand

Targeted therapies and dermatofibrosarcoma protuberans

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Oncologie

Abstract

Dermatofibrosarcoma is a rare low-grade sarcoma characterized in more than 90% of cases by (17q22; 22q13) translocation leading to a fusion transcript COL1A1-PDGFB. Surgical therapy is the standard of care; however, tyrosine-kinase inhibitors targeting PDGFR are of interest in unresectable or rare metastatic tumors. Imatinib mesylate has been approved in this indication and research is going on with other inhibitors.

Résumé

La tumeur de Darier-Ferrand ou dermatofibrosarcome protuberans est un sarcome de malignité intermédiaire de cytogénétique simple caractérisé par la présence d’une translocation chromosomique aboutissant à la fusion des chromosomes 17q22 et 22q13 et à l’expression d’une protéine de fusion COL1A1-PDGFgB qui exercerait un effet autocrine ou paracrine lors de son interaction avec le récepteur au PDGFgB présent à la surface des cellules tumorales. Son traitement repose sur la chirurgie complète, ou parfois dans des formes localement avancées, ou inopérables, ou transformées en sarcome de plus haut grade, ou métastatique sur des thérapies ciblées telles que l’imatinib mésylate (Glivec®) qui a aujourd’hui l’AMM dans cette indication, ou d’autres molécules plus récentes telles que le pazopanib qui est actuellement évalué dans le cadre d’un essai national.

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Authors and Affiliations

  1. Service de dermatologie, centre d’oncodermatologie, hôpital Saint-Louis, F-75010, Paris, France

    C. Pagès, D. Kérob & C. Lebbé

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  1. C. Pagès
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  2. D. Kérob
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  3. C. Lebbé
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Correspondence to C. Lebbé.

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Pagès, C., Kérob, D. & Lebbé, C. Thérapies ciblées et dermatofibrosarcome de Darier et Ferrand. Oncologie 15, 97–100 (2013). https://doi.org/10.1007/s10269-013-2251-4

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  • DOI: https://doi.org/10.1007/s10269-013-2251-4

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