Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans

Actas Dermosifiliogr (Engl Ed). 2018 Dec;109(10):868-877. doi: 10.1016/j.ad.2018.05.006. Epub 2018 Jul 4.
[Article in English, Spanish]

Abstract

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.

Keywords: Dermatofibrosarcoma protuberans; Diagnosis; Diagnóstico; Sarcoma; Tratamiento; Treatment.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Combined Modality Therapy
  • Dermatofibrosarcoma / diagnosis*
  • Dermatofibrosarcoma / genetics
  • Dermatofibrosarcoma / pathology
  • Dermatofibrosarcoma / therapy*
  • Humans
  • Imatinib Mesylate / therapeutic use
  • Mohs Surgery
  • Neoplasm Grading
  • Neoplasm Staging
  • Oncogene Proteins, Fusion / genetics
  • Practice Guidelines as Topic*
  • Radiotherapy, Adjuvant
  • Sarcoma / classification
  • Sarcoma / diagnosis
  • Sarcoma / pathology
  • Sarcoma / therapy
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology
  • Skin Neoplasms / therapy*

Substances

  • Antineoplastic Agents
  • COLIA1-PDGFB fusion protein, human
  • Oncogene Proteins, Fusion
  • Imatinib Mesylate