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What is a Dermatofibrosarcoma Protuberans?

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  • Written By: Malcolm Tatum
  • Edited By: Bronwyn Harris
  • Last Modified Date: 13 September 2016
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Commonly known simply as DFSP, dermatofibrosarcoma protuberans is a type of tumor that has several of the characteristics associated with fibroblasts. In most cases, this tumor develops in the skin, and has the capacity to eventually travel to the lungs, although this is a relatively rare occurrence. For the most part, dealing with a dermatofibrosarcoma protuberans tumor requires surgical removal, since other methods tend to have a very low success rate.

Like many types of tumors, a DFSP comes into being because the cells begin to divide rapidly, eventually developing into an abnormal growth. This is because a fusion of two specific genes. The collagen gene and the platelet-derived growth factor gene, take place. The resulting fusion gene does not produce protein, as would normally be the case. Instead, it produces a growth factor that spurs the unusual growth of the tumor, even though the gene itself reads this production as creating protein. At first, the tumor may appear to nothing more than a small bump under the skin. Over time, a dermatofibrosarcoma protuberans may be begin to work its way into muscle tissue, and possibly spread to surrounding organs.

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The prognosis for dermatofibrosarcoma protuberans, especially if detected early, is fairly good. There are sometimes efforts to use chemotherapy to shrink the tumor. The use of imatinib, a mild drug used in various forms of chemotherapy, can sometimes produce promising results. There is also a good chance that radiation therapy will be employed along with the chemotherapy treatments.

However, surgery is often the preferred method of dealing with a dermatofibrosarcoma protuberans. Removal of the tumor also makes it possible to examine the surrounding tissue for signs that the tumor has begun to release cells to other parts of the body. Fortunately, this procedure is relatively simple and rarely involves much in the way of complications. Even when surgery is utilized, it is not uncommon for follow-up treatments of radiation or chemotherapy to take place, simply as a precaution.

While not necessarily considered the most dangerous of all types of tumors, it is important to realize that a dermatofibrosarcoma protuberans is a serious matter. Early detection makes a significant impact on the process of treatment, as well as the duration of the recovery period. Following the instructions of your physician, both during the the process of dermatofibrosarcoma protuberans treatment via radiation and chemotherapy, as well as after surgery, will help to minimize any symptoms or discomfort that may be present.

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