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 β (PDGFβ) with the collagen type 1α1 (COL1α1) 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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Pediatric Blood & Cancer
Erasmus MC: University Medical Center Rotterdam

Gooskens, S.L.M, Oranje, A.P, van Adrichem, L.N.A, de Waard-van der Spek, F.B, den Hollander, J.C, van de Ven, C.P, & van den Heuvel-Eibrink, M.M. (2010). Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP). Pediatric Blood & Cancer, 55(2), 369–373. doi:10.1002/pbc.22494