Diagnosis and treatment of dermatofibrosarcoma protuberans. European interdisciplinary guideline - update 2024
- PMID: 39904126
- DOI: 10.1016/j.ejca.2025.115265
Diagnosis and treatment of dermatofibrosarcoma protuberans. European interdisciplinary guideline - update 2024
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').
Keywords: Dermatofibrosarcoma; Guideline.
Copyright © 2025 The Authors. Published by Elsevier Ltd.. All rights reserved.
Conflict of interest statement
Declaration of Competing Interest Dr. Brochez has nothing to disclose. Dr. Emile has nothing to disclose Dr. Forsea has nothing to disclose. Dr. Garbe reports personal fees from CeCaVa, MSD, NeraCare, and Philogen, outside the submitted work. Dr. Harwood has nothing to disclose Dr. Hauschild reports grants and personal fees from Amgen, BMS, MerckPfizer, MSD/Merck, Neracare, Philogen, Pierre Fabre, Regeneron, Sanofi-Genzyme, Replimune, and Novartis Pharma, personal fees from Curevac, Eisai, Kyowa Kirin, Highlight Therapeutics, Immunocore, Iovance, Curevac, Seagen, IO Biotech, Dermagnostix, Xenthera, Agenus, Almirall, Sunpharma, and Incyte, grants from Huya Biosciences, outside the submitted work. Dr. Italiano reports Consulting or Advisory Role for Bayer, Boehringer, Daiichy-Sankyo, Merck, MSD, Novartis, and Roche, reports research Funding (Institution) from Bayer, Beigene, BMS, Boehringer, Daiichy-Sankyo, Invox Pharma, Merck, MSD, Novartis, Pharmamar, and Roche Dr. Kandolf reports personal fees for advisory boards from Bristol Myers-Squibb, MSD, Novartis Roche, Janssen, and Abbvie, outside the submitted work. Dr. Kelleners-Smeets reports personal fees from Sanofi, Novartis, MSD, and Sun Pharma, outside the submitted work. Dr. Lallas reports personal fees from Avene, MSD, Regeneron, and BMS, outside the submitted work. Dr. Lebbe reports personal fees from MSD, Pierre Fabre, BMS, Sanofi, and Immunocore, outside the submitted work. Dr. Leiter reports personal fees from Regeneron, Sanofi, MSD, Novartis, Almirall Hermal, and Sun Pharma, outside the submitted work. Dr Llombart reports Consulting or Advisory Role from Sun Pharma, Roche pharma and Merck, outside the submitted work. Dr. Longo has nothing to disclose. Dr. Malvehy reports grants and personal fees from Almirall, ISDIN, and La Roche-Posay, personal fees from MSD, and Pierre Fabre, grants from Philogen, Castle biosciences, BMS, non-financial support and others from Vivascope, Damae medical, Canfield, and Fotofinder, outside the submitted work. Dr. Mijuskovic reports personal fees for advisory boards from Beiersdorf, Eli Lilly, Janssen, La Roche Posay, and Novartis, outside the submitted work Dr. Moreno-Ramírez has nothing to disclose. Dr. Mosterd has nothing to disclose Dr. Tagliaferri has nothing to disclose Dr. Saiag reports personal fees from Novartis, MSD, NeraCare, BMS, Pierre Fabre, Merck, personal fees and grants from Damae Medical, outside the submitted work. Dr. Vieira has nothing to disclose. Dr Ugurel reports research support from Bristol Myers Squibb and Merck Serono; speakers and advisory board honoraria from Bristol Myers Squibb, Merck Sharp & Dohme, Merck Serono, and Novartis; and meeting and travel support from Almirall, Bristol-Myers Squibb, IGEA Clinical Biophysics, Merck Sharp & Dohme, Novartis, Pierre Fabre, and Sun Pharma; outside the submitted work. Dr. Zalaudek reports personal fees from Philogen, MSD, Novartis, Sanofi, La Roche Posay, Sunpharma, Biogena, Cieffe Derma, outside the submitted work
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