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Actas Dermosifiliogr. 2018 Dec;109(10):868-877. doi: 10.1016/ Epub 2018 Jul 4.

Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans.

[Article in English, Spanish]

Author information

Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España. Electronic address:
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
Servicio de Dermatología, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, España.
Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, España.


Sarcomas comprise a broad group of tumors, many of whose biological behavior and aggressiveness differ from one type to another. The therapeutic approach is generally multidisciplinary and often complex. Developments in surgical and oncological dermatology during the last few decades have positioned dermatologists as specialists in the diagnosis and treatment of skin cancer. The aim of this article is to review the main soft tissue sarcomas that typically affect the skin. Dermatofibrosarcoma protuberans is a low-grade malignant sarcoma. It exhibits slow-growth, is locally invasive, and has low metastatic potential (<3%). Mohs micrographic surgery is the treatment of choice. The COL1A1-PDGFB translocation should be analyzed in cases of unclear diagnosis and when it is necessary to identify candidates for tyrosine kinase inhibitors. Imatinib is indicated for the treatment of locally advanced and metastatic dermatofibrosarcoma protuberans.


Dermatofibrosarcoma protuberans; Diagnosis; Diagnóstico; Sarcoma; Tratamiento; Treatment


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