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').