Access Keys:
Skip to content (Access Key - 0)

Dermatofibrosarcoma protuberans (DFSP)

  • First described in 1924 by Darier and Ferrand
  • Intermediate-grade malignancy thought to arise from dermis
  • Appears clinically as a nodular cutaneous mass that may, with time, develop satellite nodules or even nodal/visceral metastases
  • Histologically similar to solitary fibrous histiocytoma, and can be confused with plexiform neurofibroma
    • DFSP distinguished from solitary fibrous histiocytoma by diffuse CD34 positivity, and from plexiform neurofibroma by a complete absence of S100 positivity
  • 95% of DFSPs characterized by t(17;22), which fuses PDGF-beta gene to the constitutively active Col1A1 promoter, leading to overproduction of PDGF-beta locally
    • Thought to be primarily responsible for observed phenotype
  • Primary treatment is wide surgical excision
  • Tyrosine kinase inhibitor imatinib active against PDGF-beta receptor-associated tyrosine kinase; has been shown to be effective adjuvant in recurrent disease
  • Can dedifferentiate, most commonly into a high-grade fibrosarcoma; secondary fibrosarcoma arising from DFSP typically does not express CD34
  • Pigmented variant known as a Bednar tumor


Enzinger and Weiss's Soft Tissue Tumors. 4th ed. Mosby 2001.

Peer Review

OrthopaedicsOne Peer Review Workflow is an innovative platform that allows the process of peer review to occur right within an OrthopaedicsOne article in an open, transparent and flexible manner. Learn more

Instructions for Authors

Read our Instructions for Authors to learn about contributing or editing articles on OrthopaedicsOne.

Content Partner

Learn about becoming an OrthopaedicsOne Content Partner.

Academic Resources

Resources on Dermatofibrosarcoma protuberans (DFSP) from Pubget.

The license could not be verified: License Certificate has expired!
Orthopaedic Web Links

Internet resources validated by

The license could not be verified: License Certificate has expired!
Related Content

Resources on Dermatofibrosarcoma protuberans (DFSP) and related topics in OrthopaedicsOne spaces.

Page: Dermatofibrosarcoma protuberans (DFSP) (OrthopaedicsOne Articles)
Page: Myositis ossificans (OrthopaedicsOne Articles)
Page: Glomus tumor (OrthopaedicsOne Articles)
Page: Pigmented villonodular synovitis (PVNS) (OrthopaedicsOne Articles)
Page: Synovial sarcoma (OrthopaedicsOne Articles)
Page: Giant cell tumor of tendon sheath (OrthopaedicsOne Articles)
Page: Fibromatosis (OrthopaedicsOne Articles)
Page: Angiosarcoma (OrthopaedicsOne Articles)
Page: Desmoplastic fibroma (OrthopaedicsOne Articles)
Page: Epithelioid sarcoma (OrthopaedicsOne Articles)
Showing first 10 of 150 results