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Dermatofibrosarcoma Protuberans (DFSP)

Slow-growing, locally aggressive dermal sarcoma with characteristic COL1A1-PDGFB translocation.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Dermatofibrosarcoma Protuberans (DFSP)?

Dermatofibrosarcoma protuberans (DFSP) is a rare dermal soft-tissue sarcoma characterized by t(17;22) producing the COL1A1-PDGFB fusion gene and constitutive PDGFR signaling.

It usually appears as a slowly enlarging, indurated plaque or nodule on the trunk, shoulders or extremities of young adults. Distant metastases are uncommon, but local recurrence is high without adequate excision.

Diagnosis is histopathological with CD34 positivity and confirmed by molecular detection of the fusion. Imaging may be needed to assess deep extension.

Symptoms

Slowly enlarging firm plaque
Pink, brown or violaceous nodule on the skin
Multifocal, protuberant lesion over years
Pain or tenderness late in disease
Restricted mobility if deep involvement
Cosmetic disfigurement

Risk Factors

Young to middle-aged adults
Prior trauma or scar at the site (proposed)
Pregnancy may accelerate growth
Prior radiotherapy in the region
No strong inherited predisposition known

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent skin plaque growing over months
  • Recurrent lesion at a previous excision site
  • Rapid growth or pain in a known mass
  • Deep induration suggesting fascial extension
  • Postoperative surveillance after DFSP excision

Treatment Methods

01
Wide local excision with 2–4 cm margins
02
Mohs micrographic surgery for cosmetically sensitive areas
03
Imatinib (PDGFR inhibitor) for locally advanced or metastatic disease
04
Adjuvant radiotherapy for close or positive margins
05
Long-term clinical follow-up for local recurrence
06
Multidisciplinary sarcoma board for advanced cases

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dermatoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.