TY - JOUR
T1 - Diagnosis and treatment of dermatofibrosarcoma protuberans. European interdisciplinary guideline – update 2024
AU - Saiag, Philippe
AU - Lebbe, Celeste
AU - Brochez, Lieve
AU - Emile, Jean François
AU - Forsea, Ana Maria
AU - Harwood, Catherine
AU - Hauschild, Axel
AU - Italiano, Antoine
AU - Kandolf, Lidija
AU - Kelleners-Smeets, Nicole WJ
AU - Lallas, Aimilios
AU - Leiter, Ulrike
AU - Llombart, Beatriz
AU - Longo, Caterina
AU - Malvehy, Josep
AU - Mijuskovic, Zeljko
AU - Moreno-Ramirez, David
AU - Mosterd, Klara
AU - Tagliaferri, Luca
AU - Ugurel, Selma
AU - Vieira, Ricardo
AU - Zalaudek, Iris
AU - Garbe, Claus
N1 - Publisher Copyright:
© 2025 The Authors
PY - 2025/3/11
Y1 - 2025/3/11
N2 - 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').
AB - 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').
KW - Dermatofibrosarcoma
KW - Guideline
U2 - 10.1016/j.ejca.2025.115265
DO - 10.1016/j.ejca.2025.115265
M3 - (Systematic) Review article
SN - 0959-8049
VL - 218
JO - European Journal of Cancer
JF - European Journal of Cancer
M1 - 115265
ER -