title: Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP).
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.
authors.affiliation: Department of Pediatric Oncology/Hematology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.
authors.forename: Saskia L M
authors.initials: SL
authors.lastname: Gooskens
authors.forename: Arnold P
authors.initials: AP
authors.lastname: Oranje
authors.forename: Leon N A
authors.initials: LN
authors.lastname: van Adrichem
authors.forename: Flora B
authors.initials: FB
authors.lastname: de Waard-van der Spek
authors.forename: Jan C
authors.initials: JC
authors.lastname: den Hollander
authors.forename: Cees P
authors.initials: CP
authors.lastname: van de Ven
authors.forename: Marry M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents; D001549:Benzamides; D002675:Child, Preschool; D018223:Dermatofibrosarcoma / Q000188:drug therapy*; D006801:Humans; D000068877:Imatinib Mesylate; D007223:Infant; D008297:Male; D010879:Piperazines / Q000627:therapeutic use*; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines / Q000627:therapeutic use*; D012074:Remission Induction / Q000379:methods; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate