[Dermatofibrosarcoma: Management].
Copyright policy )[Dermatofibrosarcoma: Management].
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.
- University of Lille France
- Centre Oscar Lambret France
- Evaluation des technologies de santé et des pratiques médicales France
- Hôpital Claude Huriez France
- Hôpital régional Switzerland
Genetic Markers, Skin Neoplasms, Chromosomes, Human, Pair 22, Dermatofibrosarcoma, Proto-Oncogene Proteins c-sis, Fusion gene, Collagen Type I, Translocation, Genetic, [SDV] Life Sciences [q-bio], Collagen Type I, alpha 1 Chain, Margins, Mohs microsurgery, Rare Diseases, Imatinib, Humans, Neoplasm Recurrence, Local, Chromosomes, Human, Pair 17
Genetic Markers, Skin Neoplasms, Chromosomes, Human, Pair 22, Dermatofibrosarcoma, Proto-Oncogene Proteins c-sis, Fusion gene, Collagen Type I, Translocation, Genetic, [SDV] Life Sciences [q-bio], Collagen Type I, alpha 1 Chain, Margins, Mohs microsurgery, Rare Diseases, Imatinib, Humans, Neoplasm Recurrence, Local, Chromosomes, Human, Pair 17
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