Dermatofibrosarcoma Protuberans (DFSP)

DFSP

Overview

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma that develops in the deep layers of the skin. It typically presents as a firm, painless plaque or nodule that gradually enlarges over months to years. While it rarely metastasizes, DFSP is locally aggressive and has a high recurrence rate if not completely excised.

Key Symptoms

  • Firm, painless, skin-colored or reddish-brown plaque or nodule
  • Slow growth over months to years, often initially flat before becoming raised
  • Rubbery or hard texture on palpation
  • May develop multiple nodules or protuberant masses over time
  • Usually 1-5 cm at diagnosis but can grow much larger if untreated
  • Overlying skin may appear stretched, shiny, or discolored

Affected Areas

Trunk (most common), Arms, Legs, Head and neck

Care Instructions

  1. Seek evaluation by a dermatologist or surgical oncologist for any firm, slowly growing skin nodule.
  2. Mohs micrographic surgery is the preferred treatment for complete margin control.
  3. Wide local excision with adequate margins is an alternative when Mohs surgery is not available.
  4. Regular follow-up with imaging and clinical exams is essential due to risk of local recurrence.
  5. Genetic testing may reveal the COL1A1-PDGFB fusion gene characteristic of DFSP.
  6. Imatinib (a targeted therapy) may be used for unresectable or metastatic cases.

Medical Disclaimer: This information is for educational purposes only and does not replace professional medical advice. Always consult a qualified dermatologist for diagnosis and treatment.

Prevalence: Rare; approximately 1,000 new cases per year in the United States