What 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:

• 5-year survival rate = 99.2%

• 15-year survival rate = 97.2%

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 true.

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 cancer.

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 operating room.

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:

• Chemotherapy. One 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 have surgery.

Follow-up Essential
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.

More Information
Dermatofibrosarcoma Protuberans (DFSP): What It Looks Like

References:
Cooper JZ, Brown MD. “Tumors and Hyperplasias of the Dermis and Subcutaneous Fat.” In Wolff K., Goldsmith LA, Katz SI et al. [editors] Fitzpatrick’s Dermatology in General Medicine, 7^th edition. United States. McGraw Hill Medical; 2008. P. 1159-61.

Criscione VD, Weinstock MA. “Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002.” Journal of the American Academy of Dermatology June 2007; 56: 968-73.

Gloster HM, Jr. “Dermatofibrosarcoma protuberans.” Journal of the American Academy of Dermatology September 1996; 35: 355-74; quiz 75-6.

Halpern M, Chen E, Ratner D. “Sarcomas.” In Nouri K. [editor]. Skin Cancer. United States. McGraw Hill Medical; 2008. p. 217-8.

Kamino H, Meehan SA, and Pui J. “Fibrous and Fibrohistiocytic Proliferations of the Skin and Tendons.” In Bolognia JL, Jorizzo JL, Rapini RP et al. [editors] Dermatology, 2^nd edition. Spain. Mosby Elsevier. 2008. p. 1825-7.

Love WE, Keiler SA, Tamburro JE et al. “Surgical management of congenital dermatofibrosarcoma protuberans.” Journal of the American Academy of Dermatology. December 2009; 61: 1014-23.

Young CR, Albertini MJ. “Atrophic dermatofibrosarcoma protuberans: case report, review, and proposed molecular mechanisms.” Journal of the American Academy of Dermatology. October 2003; 49: 761-4.

All content solely developed by the American Academy of Dermatology