is Dermatofibrosarcoma Protuberans (DFSP)?
Dermatofibrosarcoma protuberans (DFSP)
is a rare type of skin cancer. It is considered rare because for
every one million people, about 5 to 8 will develop DFSP.
While this skin cancer tends to grow slowly, it can be aggressive.
DFSP can grow deeply into the skin. It can invade the fat, muscle,
and bone. DFSP rarely spreads to other parts of the body. This gives
DFSP a higher survival rate:
What Causes DFSP?
It is not clear what causes this type of skin cancer. A scar that
develops after a burn or surgery may increase the risk for
developing DFSP. More research is needed to learn whether this is
Who Gets DFSP?
This skin cancer develops in people of all races and ages. Some
people seem to have an increased risk:
Between the ages of 30 and 50
years. While DFSP can develop at any age, most people
develop it between these ages. DFSP is rare in children.
Had skin trauma. A scar left
by a surgery or burn may increase the risk of developing DFSP.
Women. One large-scale study
found DFSP to be more common in women. Other studies have found
that it tends to develop equally in men and women.
African Americans. One
large-scale study found that DFSP might be more common in
African Americans. Other studies have found that it develops
about equally among races.
Signs and Symptoms of DFSP
The first sign is generally a flat or slightly raised patch of skin
that feels hard to the touch. It often looks like a scar or wrinkled
patch of skin. The patch may be violet, reddish brown, or skin
colored. As DFSP grows, a tumor may appear. Sometimes more than 1
tumor appears. The tumors also tend to be violet, reddish brown, or
skin colored. By the time a tumor appears, the cancer is usually
growing more rapidly. As the cancer starts to grow more rapidly, a
tumor may open up, bleed, or become painful. But in many cases, DSFP
does not cause any discomfort or pain.
A rare form of DFSP, called a Bednar tumor, contains cells that
produce melanin. Melanin is the substance that gives skin its color.
Because of this, a Bednar tumor may contain various colors,
including red and brown. The Bednar tumor occurs more frequently in
blacks than in whites.
How is DFSP Diagnosed?
When a dermatologist suspects skin cancer, the dermatologist
performs a biopsy. This procedure can be safely performed during an
office visit. To perform a biopsy, the dermatologist will remove the
suspicious lesion, or part of it, so that it can be examined under a
microscope. This is the only way to know whether a patient has skin
Treatment for DFSP
While slow growing, treatment is important. DFSP can grow deeply
into the skin. A dermatologist will consider this and other factors
before deciding which treatment(s) is most appropriate. Other
considerations include where DFSP is located on the body and the
patient’s health. Treatment options include:
Mohs micrographic surgery (Mohs).
Because DFSP can grow deeply into the skin and even into the fat
and bone, this surgical procedure is often used to treat DFSP
today. Mohs allows the surgeon to remove one layer of skin at a
time. Each layer that is removed is placed under a microscope so
that the surgeon can look for cancer cells. This process
continues until the surgeon no longer finds cancer cells. Mohs
is often performed in a dermatologist’s office.
Because treatment continues until cancer cells are no longer
found, Mohs reduces the risk that the DFSP will return.
Excision. Mohs may not be
appropriate for every patient. If a tumor is large, DFSP may be
treated with a surgical procedure called “excision.” This
involves surgically removing DFSP and a portion of
normal-looking skin. This procedure may be performed in a
dermatologist’s office. Sometimes it needs to be performed in an
Treatment for Advanced DFSP
When a patient has advanced DFSP, the cancer has grown deeply. It
may have reached the muscle or bone. It may have spread to other
parts of the body. In these cases, more than one treatment may be
used to increase the likelihood that all of the cancer is killed or
removed. Treatment options for advanced DFSP are:
medication, imatinib mesylate, has received approval from the
U.S. Food and Drug Administration (FDA) for the treatment of
DFSP. This medication targets and turns off proteins that allow
cancer cells to grow. This medication is not meant to treat
everyone who has DFSP. It has been approved to treat an adult
who has DFSP that cannot be removed with surgery and DFSP that
keeps returning or has spread to other parts of the body.
In some patients with advanced DFSP, imatinib mesylate may be
taken before surgery. It may help reduce the size of the tumor,
which can make surgery more effective. Using both the medication
and surgery to treat advanced DFSP has been shown to greatly
reduce the risk of DFSP returning.
Radiation therapy. Because
DFSP can return after treatment, radiation therapy may be
prescribed. Radiation therapy is usually only given after
surgery when the risk of DFSP returning is high.
Radiation may be a treatment option for a patient who cannot
DSFP is a type of skin cancer that has a tendency to return after
treatment. Continuing to see a dermatologist is essential. When DFSP
returns, it often does so within 3 years of treatment. This is why
dermatologists recommend that during the first 3 years, patients
return for follow-up examinations every 3 to 6 months. If DFSP does
not return within the first 3 years, dermatologists recommend that a
patient return once a year for a thorough exam. Anyone who has had
DFSP should have these yearly exams for life.
Protuberans (DFSP): What It Looks Like
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Young CR, Albertini MJ. “Atrophic dermatofibrosarcoma protuberans:
case report, review, and proposed molecular mechanisms.” Journal
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content solely developed by the American Academy of Dermatology
DFSP usually looks like a scar or wrinkled patch of skin
that feels hard to the touch.
Photograph used with permission of the Journal
of the American Academy of Dermatology.
The photograph was published in the Journal
of the American Academy of Dermatology,
Vol. # 49,
Young CR, Albertini MJ,
“Atrophic dermatofibrosarcoma protuberans:
case report, review, and proposed molecular mechanisms.”
761-4. Copyright Elsevier (2003).
Journal of the American Academy of Dermatology.