Abstract
Aim:Dermatofibrosarcoma protuberans (DFSP) accounts for less than 2% of all soft-tissue sarcomas.Patients & methods:We retrospectively reviewed our database for patients with locally advanced or metastatic DFSP who had presented to our clinic between January 2016 and January 2020.Results:We identified a total of 14 patients, of whom ten had sarcomatous transformation. Eleven cases had metastatic disease and three were locally advanced. The initial partial response rate to first-line imatinib was 76.9% and the overall median progression-free survival on imatinib was 15 months.Conclusion:We had a high proportion of patients with sarcomatous transformation, in contrast to their relative rarity in the West. While most patients had initial good responses to imatinib, second-line therapies were not as effective.
Highlights
Imatinib is the drug of choice for use in the management of unresectable, recurrent or metastatic Dermatofibrosarcoma protuberans (DFSP)
There is no significant difference in clinical outcomes with either 400 or 800 mg of imatinib
The initial partial response rate to first-line imatinib was 76.9% (10/13) and the overall median progression-free survival on imatinib was 15 months at a median follow-up of 17.5 months
Summary
Dermatofibrosarcoma protuberans (DFSP) accounts for less than 2% of all soft-tissue sarcomas
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