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Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP)
Saskia L M Gooskens1, Arnold P Oranje, Leon N A van Adrichem
1Department of Pediatric Oncology/Hematology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.
Pediatric Blood & Cancer
|June 29, 2010
Summary
Dermatofibrosarcoma protuberans (DFSP) in children is a rare soft tissue tumor. Targeted therapy with imatinib mesylate showed successful treatment outcomes in a small pediatric series.
Area of Science:
- Oncology
- Pediatric Oncology
- Molecular Oncology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor affecting children.
- DFSP is characterized by a specific gene fusion (PDGFbeta-COL1alpha1), similar to adult cases.
- This genetic hallmark suggests potential responsiveness to targeted therapies.
Observation:
- This report details the first small series of pediatric DFSP cases.
- The study observed the treatment outcomes in these young patients.
- The focus was on the efficacy of specific targeted therapies.
Findings:
- The pediatric DFSP cases exhibited a positive response to imatinib mesylate.
- Imatinib mesylate, a tyrosine kinase inhibitor, was utilized as a targeted therapy.
- The response to imatinib mesylate was a key factor in successful treatment.
Implications:
- This study suggests imatinib mesylate can be an effective treatment for pediatric DFSP.
- The findings support the use of targeted therapy in managing this rare pediatric cancer.
- Further research into tyrosine kinase inhibitors for pediatric DFSP is warranted.
