Dermatofibrosarcoma Protuberans-Derived Fibrosarcoma: Clinical History, Biological Profile and Sensitivity to Imatinib

Int J Cancer. 2011 Oct 1;129(7):1761-1762, S Stacchiotti, F Pedeutour, T Negri, E Conca, A Marrari, E Palassini, P Collini, F Keslair, C Morosi, A Gronchi, S Pilotti, PG Casali

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Abstract

Abstract

Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the COL1A1/PDGFB fusion-gene, responsible for platelet derived growth factor beta receptor (PDGFRB) activation. Fibrosarcomatous (FS) transformation in DFSP rarely occurs. The fusion-gene and PDGFRB expression/activation pattern and imatinib role in DFSP-derived FS is less defined. We reviewed all consecutive patients operated for localized DFSP at our institution from 1994 to 2009, selecting cases with FS component. We also...

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