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Updated: Oct 14, 2025
Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
Advanced dermatofibrosarcoma protuberans: an updated analysis of cases from an Indian sarcoma clinic
Azgar A Rasheed1, Adarsh Barwad2, Ekta Dhamija3
1Department of Medical Oncology, BRA-IRCH, All India Institute of Medical Sciences, New Delhi, 110029, India.
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.
Insights
Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue sarcoma. This study found a high rate of sarcomatous transformation in DFSP patients, with good initial response to imatinib but limited efficacy of second-line therapies.
Area of Science:
- Oncology
- Dermatology
- Soft Tissue Sarcoma Research
Background:
- Dermatofibrosarcoma protuberans (DFSP) represents a rare subset of soft-tissue sarcomas, accounting for less than 2% of all cases.
- Understanding the clinical characteristics and treatment outcomes of DFSP, particularly advanced or metastatic forms, is crucial for improving patient management.
Purpose of the Study:
- To retrospectively analyze patients with locally advanced or metastatic DFSP treated between January 2016 and January 2020.
- To evaluate the efficacy of first-line imatinib therapy and the impact of sarcomatous transformation on treatment outcomes in DFSP.
Main Methods:
- Retrospective review of a database for patients diagnosed with locally advanced or metastatic DFSP.
- Inclusion criteria: patients presenting to the clinic between January 2016 and January 2020.
- Analysis of treatment response, progression-free survival, and characteristics including sarcomatous transformation.
Main Results:
- A total of 14 DFSP patients were identified, with 10 exhibiting sarcomatous transformation.
- Eleven patients had metastatic disease, and three had locally advanced disease.
- First-line imatinib achieved a 76.9% partial response rate, with a median progression-free survival of 15 months.
Conclusions:
- The study observed a higher proportion of sarcomatous transformation in DFSP compared to Western populations.
- While imatinib showed good initial efficacy, subsequent therapies demonstrated limited effectiveness.
- Further research into optimal treatment strategies for advanced DFSP, especially with sarcomatous transformation, is warranted.
