Advanced dermatofibrosarcoma protuberans: an updated analysis of cases from an Indian sarcoma clinic

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...

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Detalles Bibliográficos
Autores principales: Rasheed, Azgar A, Barwad, Adarsh, Dhamija, Ekta, Garg, Rakesh, Pandey, Rambha, Shamim, Shamim A, Arun Raj, Sreedharan T, Rastogi, Sameer
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Future Science Ltd 2021
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8558865/
https://www.ncbi.nlm.nih.gov/pubmed/34737884
http://dx.doi.org/10.2144/fsoa-2020-0198
Descripción
Sumario: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.

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