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2010•Pediatric Blood & CancerRequires access

Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP)

Saskia L. Gooskens, Arnold P. Oranje, Léon N. A. van Adrichem, Flora B. de Waard‐van der Spek, Jan C. den Hollander, Cornelis P. van de Ven, Marry M. van den Heuvel‐Eibrink

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Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor in children. DFSP is characterized by a specific fusion of the platelet-derived growth factor beta (PDGFbeta) with the collagen type 1alpha1 (COL1alpha1) gene which renders these tumors responsive to targeted therapy with tyrosine kinase inhibitors, such as imatinib mesylate, as is reported in adults. In the current report, we describe the first small pediatric DFSP series, in which response to imatinib mesylate contributed to successful treatment outcome.

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What this paper is about

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor in children. DFSP is characterized by a specific fusion of the platelet-derived growth factor beta (PDGFbeta) with the collagen type 1alpha1 (COL1alpha1) gene which renders these tumors responsive to targeted therapy with tyrosine kinase inhibitors, such as imatinib mesylate, as is reported in adults. In the current report, we describe the first small pediatric DFSP series, in which response to imatinib mesylate contributed to successful treatment outcome.

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OpenAlex reports 72 citations for this work. Citation counts describe recorded attention and do not establish research quality.

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Available abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor in children. DFSP is characterized by a specific fusion of the platelet-derived growth factor beta (PDGFbeta) with the collagen type 1alpha1 (COL1alpha1) gene which renders these tumors responsive to targeted therapy with tyrosine kinase inhibitors, such as imatinib mesylate, as is reported in adults. In the current report, we describe the first small pediatric DFSP series, in which response to imatinib mesylate contributed to successful treatment outcome.

Key concepts: Dermatofibrosarcoma protuberans, Imatinib mesylate, Medicine, Dermatofibrosarcoma, Imatinib, Tyrosine-kinase inhibitor, Tyrosine kinase, Dermatology

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