Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that develops from the dermis (second layer of the skin) and can grow slowly for years before detection. Although DFSP rarely spreads to distant sites, it is locally aggressive and can recur if not fully removed.
At Aglow Dermatology in New York City, board-certified dermatologist Dr. Dina M. Strachan, MD, provides dermatologic care for complex skin conditions, including rare cancers such as DFSP.
Here is what you need to know about DFSP:
What is Dermatofibrosarcoma Protuberans (DFSP)?
DFSP is a soft tissue sarcoma (type of cancer that develops from connective tissue) originating from the dermis. It typically presents as a firm, painless plaque or nodule that slowly enlarges over time. There is a variant of DFSP that is more pigmented called the Bednar tumor. Though rare, it can become more invasive if diagnosis is delayed.
Who Is Most Commonly Affected by DFSP?
This tumor most frequently occurs in adults aged 20–50 and is slightly more common in men. Although they occur in all skin types, DFSP and Bednar tumors have a higher incidence in Black individuals compared to white populations.
How It May Appear in Skin of Color
In skin of color DFSP may appear more brown or purple rather than reddish. They may have the appearance of keloids, which are more common in Black and Hispanic people, leading to delayed diagnosis.
What Are the Symptoms and Appearance of DFSP?
Commonly found on the trunk, and sometimes the limbs, head or neck DFSP tends to be painless. The lesions are firm and raised, or may have a deep nodular component. The tumor may ulcerate or bleed in late stages.
What Causes DFSP and Who Is at Risk?
Genetic mutations after birth cause DFSP. It is not inherited from family. Other risk factors may include prior trauma to the site (e.g., surgical scars, tattoos). This is another reason it can be confused with a keloid. Men and African Americans have a higher risk.
How Is DFSP Diagnosed?
DFSP is diagnosed first by clinical examination by a dermatologist. This is followed by a punch biopsy and histological examination by a dermatopathologist. Once the diagnosis is confirmed, imaging may be ordered to determine tumor depth and involvement. Genetic testing can also confirm the diagnosis and help with determining the prognosis with respect to recurrence and response to treatment.
Treatment Options for DFSP
Surgical treatment is the standard of care for DFSP and this may be either wide local excision or tissue sparing Mohs surgery. When surgery is not an option radiation therapy is an alternative as is targeted therapy with Imatinib.
Can advanced DFSP Be Prevented or Treated Early?
Yes, skin exams and biopsies of slow-growing, firm nodules help catch DFSP and Bednar tumors early. Early surgical intervention greatly reduces recurrence. Consider a biopsy in cases when presumed keloids are not responding to treatment
Frequently Asked Questions
It is rarely life-threatening if treated properly, but can be disfiguring and aggressive locally.
Yes, recurrence rates can be high if not fully excised—especially with wide local excision without Mohs.
Yes and no. It is caused by a genetic mutation after birth but it is not inheirited from family. Most cases are sporadic. Genetic testing is not standard unless targeted therapy is being considered.
When to See a Dermatologist
If you have a persistent, firm nodule or plaque—especially one growing over time—you should see a board-certified dermatologist for a skin cancer screening. Early diagnosis allows for less invasive treatment and better cosmetic outcomes. Schedule a consultation with Dr. Strachan »