TY - JOUR
T1 - Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP)
AU - Gooskens, Saskia L.M.
AU - Oranje, Arnold P.
AU - Van Adrichem, Leon N.A.
AU - De Waard-Van Der Spek, Flora B.
AU - Den Hollander, Jan C.
AU - Van De Ven, Cees P.
AU - Van Den Heuvel-Eibrink, Marry M.
PY - 2010/8
Y1 - 2010/8
N2 - 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.
AB - 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.
KW - Children
KW - Dermatofibrosarcoma protuberans
KW - Imatinib
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=77955104531&partnerID=8YFLogxK
U2 - 10.1002/pbc.22494
DO - 10.1002/pbc.22494
M3 - Article
C2 - 20582941
AN - SCOPUS:77955104531
SN - 1545-5009
VL - 55
SP - 369
EP - 373
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
IS - 2
ER -