[Dermatofibrosarcoma: Management]

Bull Cancer. 2018 Nov;105(11):1094-1101. doi: 10.1016/j.bulcan.2018.08.008. Epub 2018 Oct 5.
[Article in French]


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.

Keywords: Dermatofibrosarcoma; Dermatofibrosarcome; Fusion gene; Imatinib; Marges; Margins; Microchirurgie de Mohs; Mohs microsurgery; Transcrit de fusion.

Publication types

  • Review

MeSH terms

  • Chromosomes, Human, Pair 17 / genetics
  • Chromosomes, Human, Pair 22 / genetics
  • Collagen Type I / genetics
  • Collagen Type I, alpha 1 Chain
  • Dermatofibrosarcoma* / diagnostic imaging
  • Dermatofibrosarcoma* / genetics
  • Dermatofibrosarcoma* / pathology
  • Dermatofibrosarcoma* / therapy
  • Genetic Markers
  • Humans
  • Neoplasm Recurrence, Local
  • Proto-Oncogene Proteins c-sis / genetics
  • Rare Diseases* / diagnostic imaging
  • Rare Diseases* / genetics
  • Rare Diseases* / pathology
  • Rare Diseases* / therapy
  • Skin Neoplasms* / diagnostic imaging
  • Skin Neoplasms* / genetics
  • Skin Neoplasms* / pathology
  • Skin Neoplasms* / therapy
  • Translocation, Genetic


  • Collagen Type I
  • Collagen Type I, alpha 1 Chain
  • Genetic Markers
  • Proto-Oncogene Proteins c-sis