Dermatofibrosarcoma Protuberans (DFSP)

Dermatofibrosarcoma Protuberans (DFSP) is a rare type of cancer, a soft tissue sarcoma that develops in the deep layers of skin. It is sometimes described as having tentacles that can grow into surrounding fat, muscle and even bone. DFSP is most commonly found on the torso, but can also be seen on the arms, legs, head and neck. It has a tendency to recur in the same location after it is removed but only spreads to other parts of the body in about 5 percent of cases.

DFSP most often starts as a small, firm patch of skin, approximately one to five centimeters in diameter. The skin is occasionally flat or depressed. It can be purplish, reddish or flesh-colored. The tumor typically grows very slowly (over months to years) and can become a raised nodule.

It tends to affect people between the age of 20 and 50, but it has been diagnosed in people of all ages. There are about 1,000 cases diagnosed in the United States each year, with an incidence of about one to five people per million. The tumors affect African American patients about twice as often as Caucasian, patients.

Treatment

Many cases of DFSP can be treated by a dermatologist who routinely diagnoses and treats lesions of the skin. However, in cases of large or advanced DFSPs, or when major reconstructive surgery is indicated, a multidisciplinary approach may be necessary. Our team may involve a surgeon, pathologist, radiologist, oncologist, radiation oncologist and plastic surgeon for reconstruction.

Treatment options include:

Excision

A surgical procedure may be utilized if a tumor is large in size. It involves surgically removing the DFSP and a portion of normal-looking skin. Since these tumors have a high propensity for recurring locally, comprehensive assessment of the excision margin is important. This assessment can be challenging and requires careful, expert pathology.

In cases of advanced DFSP, the cancer has grown deeply and may have reached muscle or bone, and may even have spread to other parts of the body. This is rare, but it may require more than one treatment to effectively eradicate the cancer.

Treatment options for advanced DFSP include:

Systemic therapy

The targeted therapy imatinib has not been approved by the U.S. Food and Drug Administration for the treatment of DFSP, but is being tested in clinical trials. It targets and turns off proteins that allow certain types of cancer cells to grow. It is not meant to treat every person with DFSP, only those with DFSP that cannot be removed with surgery or DFSP that keeps returning or has spread to other parts of the body. Using both the medication and surgery to treat advanced DFSP may help reduce the risk of DFSP returning.

Radiation therapy

In a few cases, radiation treatment may be used for DFSP. Although surgery is the mainstay, radiation may be used if surgery is incomplete, if DFSP recurs or if surgery is not possible.