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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},
}