Dermatofibrosarcoma Protuberans

Eileen Bailey Health Guide February 14, 2013
  • Dermatofibrosarcoma Protuberans (DFSP) is a rare form of skin cancer that grows in the deep layers of the skin. It is most commonly found on the torso but can sometimes be found on the legs, arms, head or neck.  It is considered an aggressive type of cancer although it doesn’t usually spread to other parts of the body it does have a high recurrence rate.

     

    Appearance


    DFSP usually begins as a small, firm patch of skin and can be purple, red or flesh colored and can be either flat or slightly raised. This type of cancer grows very slowly but can grow into fat, muscle and bone surrounding the tumor, similar to tentacles or fingers. As the cancer grows, small tumors can appear, which may open up, bleed and become painful. Usually, though, there is no pain or discomfort from this type of cancer.

     

    A Bednar tumor, most often seen in people of color, is a rare type of tumor sometimes found in DFSP. This type of tumor contains melanin, which is the substance that gives skin its color. These types of tumors may have several different colors.

     

    Risk Factors


    This type of cancer can be found in all races and ages although there are some groups that may have an increased risk:

    • African Americans – Most studies have found this type of cancer to develop equally in all races but one study showed it occurring more often in African Americans
    • Women – Again, studies have shown that men and women have an equal chance of developing DFSP, however, one study showed women were more at risk
    • Skin trauma – this cancer may occur at the site of a scar from previous skin trauma or a burn
    • Age – DFSP can occur at any age, however, it is very rare in children and is most often seen in those between the ages of 30 and 50

    According to the National Institute of Health, DFSP occurs anywhere between 1 in every 100,000 to 1 in every 1,000,000 people per year.

     

    Diagnosis


    Because there is often no pain or discomfort associated with DFSP it can be ignored until it slowly becomes larger. It may be mistaken for another skin condition. Changes in your skin, especially a scar, should be checked by a dermatologist. Frequently, this type of cancer isn’t diagnosed until larger lesions or tumors develop. By this time, the cancer is often growing rapidly. It is important to have annual check-ups with your dermatologist as DFSP can be caught in the early stage during routine check-ups.

     

    If your doctor suspects cancer, he will remove a part or all of the lesion and examine it under a microscope. If it is determined that it is cancer, your doctor may order an MRI to determine the extent of tumor.

     

    Treatment


    Treatment would depend on how deep the tumor has spread. For cancers which are caught early, Mohs surgery, which removes one layer of skin at a time until no sign of cancer is present. Larger tumors may require surgical excision.

    If the cancer has grown and attached to muscles or bone or continues to recur, more aggressive treatments, such as surgery combined with the medication Imatnib may be used.  When other treatments have failed, the cancer continues to recur or surgery is not an option, radiation therapy may be used.

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    Most people recover from DFSP. The Liddy Shriver Sarcoma Initiative states the cure rate is 98 percent, in part because of the use of Mohs surgery.  For those with more advanced cancer that has metastasized, use of Imatinib has increased the rates of recovery.

     

    References:

     

    “Dermatofibrosarcoma Protuberans,” Date Unknown, Staff Writer, New York Presbyterian Hospital

     

    “What is Dermatofibrosarcoma Protuberans?” Date Unknown, Staff Writer, The Liddy Shriver Sarcoma Initiative

     

    “What is Dermatofibrosarcoma Protuberans (DFSP)?” Date Unknown, Staff Writer, SkinCancer.net