Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing skin cancer. People with DFSP have a highly favorable outlook, provided they can treat the cancer before it spreads.
Dermatofibrosarcoma protuberans (DFSP) is a rare type of cancer that begins in the middle layer of your skin called the dermis.
DFSP most often occurs in adults between ages 20 and 50, but it can affect people of all ages. The tumor typically grows slowly and rarely spreads to other parts of the body.
However, it can eventually grow into surrounding fat, muscle, and even bone tissue, making it difficult to treat and affecting your outlook.
Keep reading to learn about DFSP, its symptoms, diagnosis, treatment options, and the outlook for people with this condition.
What are the symptoms of dermatofibrosarcoma protuberans?
DFSP usually starts as a small, firm patch of skin that can feel rubbery or hard to the touch. It can look like a dermatofibroma , a harmless and common skin growth.
Over time, the patch can grow and form a raised lump (a protuberan) due to the middle layer of the skin pushing on the top layer.
Common symptoms of DFSP include:
- a small bump or patch on the skin that grows slowly
- a firm, raised nodule that can be purple, red, or brown
- pain or tenderness around the affected area
- bleeding from the affected area, though this is rare
Because DFSP grows slowly, it often takes years for people to seek medical attention, which can delay diagnosis and treatment.
Pictures of dermatofibrosarcoma protuberans
Dermatofibrosarcoma protuberans can appear as a firm, discolored lump on skin, usually less than 2 inches (5 centimeters) wide. (BSIP SA / Alamy Stock Photo)
Dermatofibrosarcoma protuberans may spread, causing a larger lump or cluster of raised lumps on the skin. (BSIP SA / Alamy Stock Photo)
In rare circumstances, dermatofibrosarcoma protuberans can grow to become quite large without treatment. (BSIP SA / Alamy Stock Photo)
How do doctors diagnose dermatofibrosarcoma protuberans?
Diagnosing DFSP typically involves several steps .
A doctor will start with a physical exam and medical history. They’ll likely recommend a skin biopsy to confirm the diagnosis. During a biopsy, they’ll remove a small sample of the affected skin tissue and examine it under a microscope.
Doctors may use different types of biopsies, for example:
- Core needle biopsy: Doctors use a medium-sized needle to extract a column of tissue.
- Excisional biopsy: A surgical procedure in which a surgeon removes an entire lump for examination.
In addition to a biopsy, doctors may recommend imaging tests like MRI or CT scans to determine the extent of the tumor and check for any possible spread to nearby tissues.
What is the treatment for dermatofibrosarcoma protuberans?
The main treatment for DFSP is surgery. The goal is to remove the entire tumor along with some of the healthy tissue around it. Otherwise, the tumor may grow back (recur). The two main surgical techniques doctors use are:
- Wide local excision: A surgeon uses a scalpel to cut out the tumor along with healthy tissue around it.
- Mohs micrographic surgery: A surgeon removes the cancerous cells layer by layer, examining each layer under a microscope until no tumor cells remain. This procedure takes a lot of time but helps preserve as much healthy tissue as possible.
The National Comprehensive Cancer Network (NCCN) recommends a margin (the area of healthy tissue around the tumor) of 2 to 4 centimeters (cm) .
In some cases, additional treatments may be necessary, such as:
- Radiation therapy: A doctor may recommend radiation therapy if the tumor is in a location that makes surgery difficult or if some cancer cells remain after surgery.
- Targeted therapy: Targeted therapy , such as imatinib (Gleevec), targets specific molecules involved in the growth of cancer cells. Doctors may recommend this for advanced DFSP or when surgery is not an option.
Follow-up care is crucial after treatment to detect the first signs of recurrence as soon as possible. Your healthcare team will likely schedule regular checkups and show you how to do self-exams.
What is the outlook for people with dermatofibrosarcoma protuberans?
The outlook for people with DFSP is generally good, especially with early detection and surgical removal. DFSP rarely spreads to other parts of the body. The overall 10-year survival rate for DFSP is 99% .
However, recurrence is common. Almost half of people with DFSP have their cancer return after a simple excision. Research suggests that a wider excision margin significantly improves your outlook.
In cases where DFSP is more advanced (spread to other tissues), the outlook can vary. Some patients may require additional treatments, such as radiation or targeted therapy.
Life expectancy for people with DFSP that has spread is about 2 years after diagnosis.
What causes dermatofibrosarcoma protuberans?
The exact cause of DFSP is not well understood, but researchers believe it’s linked to genetic changes in the skin cells.
One common genetic change associated with DFSP is a translocation (breaking and re-attachment of a piece of a chromosome to a different chromosome) between chromosomes 17 and 22. This change leads to the production of a protein that triggers the growth of cancer cells.
Who is at risk of dermatofibrosarcoma protuberans?
Several risk factors may increase the likelihood of developing DFSP, including:
- Age: Most DFSP diagnoses occur in adults between the ages of 20 and 50 years .
- Ethnicity: DFSP is more common in African Americans.
- Previous skin injuries: DFSP can develop in the sight of a previous skin injury, such as a burn, surgery, or a tattoo .
Frequently asked questions
How serious is dermatofibrosarcoma protuberans?
DFSP is a serious condition because it is a form of cancer. However, it grows slowly and is usually not life threatening with early treatment.
However, left untreated, DFSP can damage the surrounding tissues. Life expectancy after DFSP spreads is typically about 2 years .
Can dermatofibrosarcoma protuberans spread?
DFSP typically does not metastasize, but it’s possible. When it grows to invade surrounding tissues, complete surgical removal can be challenging.
Regular follow-up care after treatment is important to monitor for any signs of recurrence or spread.
Can dermatofibrosarcoma protuberans kill you?
In very rare cases, untreated DFSP can spread into distant tissues and organs ( metastasis ). Metastatic DFSP can be potentially life threatening.
However, in a 2023 study of 7,567 patients with DFSP, only 89 died from this condition.
Takeaway
DFSP is a rare but treatable type of skin cancer. Early detection and prompt treatment are key to managing this condition effectively. If you notice any unusual skin growths or changes, be sure to see a dermatologist as soon as possible.
Surgical removal is the main treatment, and the outlook is generally good. Regular follow-ups are important to ensure that the cancer does not recur.
Understanding DFSP and staying informed about your treatment options can help you take control of your health and well-being.
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