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@ARTICLE{Saiag:298572,
      author       = {P. Saiag and C. Lebbe and L. Brochez and J.-F. Emile and A.
                      M. Forsea and C. Harwood and A. Hauschild and A. Italiano
                      and L. Kandolf and N. W. Kelleners-Smeets and A. Lallas and
                      U. Leiter and B. Llombart and C. Longo and J. Malvehy and Z.
                      Mijuskovic and D. Moreno-Ramirez and K. Mosterd and L.
                      Tagliaferri and S. Ugurel$^*$ and R. Vieira and I. Zalaudek
                      and C. Garbe},
      title        = {{D}iagnosis and treatment of dermatofibrosarcoma
                      protuberans. {E}uropean interdisciplinary guideline - update
                      2024.},
      journal      = {European journal of cancer},
      volume       = {218},
      issn         = {0959-8049},
      address      = {Amsterdam [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2025-00289},
      pages        = {115265},
      year         = {2025},
      abstract     = {Dermatofibrosarcoma protuberans (DFSP) is a cutaneous
                      fibroblastic tumour that is locally aggressive, with a
                      tendency for local recurrence, but rarely metastasizes. A
                      collaboration of multi-disciplinary experts from the
                      European Association of Dermato-Oncology (EADO), the
                      European Dermatology Forum (EDF), the European Union of
                      Medical Specialists (UEMS) and the European Academy of
                      Dermatology and Venereology (EADV) was formed to update
                      recommendations on DFSP diagnosis and treatment, based on
                      current literature reviews and the experts' consensus.
                      Diagnosis is suspected clinically and confirmed by pathology
                      report, which should specify whether a transformation in
                      higher-grade fibrosarcoma occurred. Detection of specific
                      chromosomal translocations and/or fusion gene transcripts is
                      useful to confirm diagnosis. Treatment is mainly surgical,
                      intending to achieve complete resection of the tumour. To
                      reduce the recurrence rate, the treatment of choice in DFSP
                      is micrographically controlled surgery. Standard excision
                      with a lateral safety margin of 2-3 cm is an acceptable
                      alternative where only standard histopathological procedures
                      are available. Imatinib is approved in Europe for treating
                      inoperable primary tumours, locally inoperable recurrent
                      disease, and metastatic DFSP. Use of imatinib has also been
                      reported in extensive, difficult-to-operate tumours for
                      preoperative reduction of tumour size, but clinical trials
                      or large register data are required to confirm the
                      usefulness of this approach. Therapeutic decisions for
                      patients with fibrosarcomatous DFSP should be primarily made
                      by an interdisciplinary oncology team ('tumour board').},
      subtyp        = {Review Article},
      keywords     = {Dermatofibrosarcoma (Other) / Guideline (Other)},
      cin          = {ED01},
      ddc          = {610},
      cid          = {I:(DE-He78)ED01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39904126},
      doi          = {10.1016/j.ejca.2025.115265},
      url          = {https://inrepo02.dkfz.de/record/298572},
}