Dermatofibrosarcoma: management
Document type :
Article dans une revue scientifique: Article original
PMID :
Permalink :
Title :
Dermatofibrosarcoma: management
Author(s) :
Penel, Nicolas [Auteur]
METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694
El Bedoui, Sophie [Auteur]
Robin, Yves-Marie [Auteur]
Decanter, Gauthier [Auteur]
METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694
El Bedoui, Sophie [Auteur]
Robin, Yves-Marie [Auteur]
Decanter, Gauthier [Auteur]
Journal title :
Bulletin du cancer
Abbreviated title :
Bull Cancer
Publication date :
2018-10-05
ISSN :
1769-6917
English keyword(s) :
Imatinib
Dermatofibrosarcoma
Fusion gene
Mohs microsurgery
Margins
Dermatofibrosarcoma
Fusion gene
Mohs microsurgery
Margins
HAL domain(s) :
Sciences du Vivant [q-bio]
English abstract : [en]
Dematofibrosarcoma protuberans (DFSP) are very rare (1 to 4 incident cases per million of inhabitants). The local spreading of DFSP is underestimated. The histological diagnosis is challenging but we now know a specific ...
Show more >Dematofibrosarcoma protuberans (DFSP) are very rare (1 to 4 incident cases per million of inhabitants). The local spreading of DFSP is underestimated. The histological diagnosis is challenging but we now know a specific marker (translocation t(17;22)(q22;q13) (COL1A1;PDGFB)). The risk of metastatic relapse is low (and related to fibrosarcoma component); the risk of local relapse depends on the quality of surgery. Management of localized DFSP is based on large resection with meticulous analysis of margins (with or without Mohs microsurgery). Advanced stages not amenable to surgery or metastatic DFSP (with presence of COL1A1;PDGFB) are best treated with imatinib. Locally advanced DFSP potentially amenable to curative intent surgery could be treated with imatinib as neo-adjuvant treatment. The management of these tumours requires multidisciplinary expertise.Show less >
Show more >Dematofibrosarcoma protuberans (DFSP) are very rare (1 to 4 incident cases per million of inhabitants). The local spreading of DFSP is underestimated. The histological diagnosis is challenging but we now know a specific marker (translocation t(17;22)(q22;q13) (COL1A1;PDGFB)). The risk of metastatic relapse is low (and related to fibrosarcoma component); the risk of local relapse depends on the quality of surgery. Management of localized DFSP is based on large resection with meticulous analysis of margins (with or without Mohs microsurgery). Advanced stages not amenable to surgery or metastatic DFSP (with presence of COL1A1;PDGFB) are best treated with imatinib. Locally advanced DFSP potentially amenable to curative intent surgery could be treated with imatinib as neo-adjuvant treatment. The management of these tumours requires multidisciplinary expertise.Show less >
Language :
Anglais
Audience :
Internationale
Popular science :
Non
Administrative institution(s) :
CHU Lille
Université de Lille
Université de Lille
Submission date :
2019-12-09T18:19:30Z