Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma Protuberans – General Information

Dermatofibrosarcoma Protuberans is a rare malignant tumor (rare neoplasm; the neoplasia is an abnormal proliferation of the cells in a tissue or organ) which develops in the dermis, which is the second layer of the skin. Dermatofibrosarcoma Protuberans is a rare medical disorder which develops predominantly in males and it can appear at any age but it is more frequently seen in adults. The cause of this medical disorder is unknown, but researchers have discovered that in the majority of the cases, the tumor have the chromosomal translocation. The translocation makes the fuse between the collagen gene (COL1A1) and the platelet derived growth factor gene. The fibroblast (which is the origin cell of the tumor) expresses the fusion genes believing that it codes the collagen. The resulting protein is a fusion protein which will be processed into a mature platelet derived growth factor. The fibroblast contains the receptor for the growth factor and although the cells think that they produce the structural protein, in fact they produce the self stimulatory growth signal, leading to a rapidly division of the cell, and to the formation of a tumor. When the doctors analyze the tumors through a microscope they can see that the tumors are composed of spindle cells. There have been described many histologic variants of Dermatofibrosarcoma Protuberans, such as: Cellular, Aneurysmal, Angiomatoid, Atypical (also known as Pseudosarcomatous), Palisading, Plexiform, Epithelioid, Atrophic, Ossifying, with osteoclastic like giant cells, cholesterotic (this is associated with hyperlipoproteinemia), with granular cells, with Monster cells, with smooth muscle.

Dermatofibrosarcoma Protuberans – Symptoms

It is well known the fact that any medical disorder (including Dermatofibrosarcoma Protuberans) can trigger a variety of symptoms. Therefore, some of this medical condition's most uncommon symptoms have not been listed (mentioned) here. This is why we strongly recommend you to contact your personal health care provider whenever you develop any unusual, bothersome or unpleasant symptoms. Your personal physician will be able to tell you if you are suffering from Dermatofibrosarcoma Protuberans or from any other medical disorder. Here is a list of the most common symptoms that a person who suffers from this disorder, may experience: is a slow growing tumor which usually develops on the chest, abdomen, back but it may also develop on the head, neck, hands, feet, legs, hands. The tumor appears as a small bump at the surface of the skin and in some cases the bump has a red-brown or a red-blue color. The tumor begins after that to develop and to grow in the dermis to a noticeable mass, which begins to swell and bulge; the area also begins to be tender and to bleed because the skin above it is stretched. The good news is that this disease usually does not spread to all of the body leading to metastasis. This disease also presents plaques and nodules which usually feel rubbery or firm. The tumor may appear as a soft depressed zone of the skin which can make the diagnoseis of the patients even more difficult. This disease is usually diagnosed in the growth period, as then there appear large lesions.

Dermatofibrosarcoma Protuberans -  Treatment

Dermatofibrosarcoma Protuberans has an initial growth phase which is very slow and this is why tit can take a few years before the tumor begins to be visible and cause discomfort. To diagnose Dermatofibrosarcoma Protuberans the doctor or personal health care provider makes a biopsy by taking a sample of the affected tissue and sends it to the laboratory for further research and examination under a microscope. The laboratory tests reveal if the cells and the growth pattern from the sample taken by the doctor are characteristic of the Dermatofibrosarcoma Protuberans. The persons who are asymptomatic may be given the diagnostic delayed, as the pain and the redness of the skin do not appear in all of the cases and are usually taken for other skin medical condition. This is why a skin biopsy is necessary to confirm the diagnostic.

Usually the Dermatofibrosarcoma Protuberans is treated with wide excision; this means that the tumor and a large size of the tissue which surrounds the tumor are removed. Another method of treatment is the surgery called Mohs’ microscopically controlled surgery (which is also known as Mohs’ micrographic surgery). During this procedure the tumor is removed and then the doctor continues to remove the edges of the tissue which is around the tumor. After each bit of removed tissue, this is examined microscopically in order to see if there are any malignant cells. The doctor operating will continue to remove the tissue until there are no malignant cells left.

The bad news is that the Dermatofibrosarcoma Protuberans usually has a rate of recurrence which is pretty high because the tumor may have projections beyond the principal mass which is removed. But the Mohs’ surgery is effective in reducing the rate of recurrence. It is well known that the chemotherapy is not effective because the growth rate of the tumor is very slow, but in some cases radiation therapy can be recommended. The tumor can extend to metastasis only in very few cases, and the recurrences appear only in 20% of the cases and this only after three years after the surgery, this is why it is important to go to the therapy which follows the surgery.
Here is a list of drugs which the doctors may prescribe to the patients who are suffering from Dermatofibrosarcoma Protuberans:

• Gleevec – this drug interferes in the growth of some of the cancer cells. It is used to treat a type of the blood cancer (named chronic myeloid leukemia –CML- or Philadelphia chromosome positive chronic myeloid leukemia), certain stomach tumor or digestive system tumors, and also in the treatment of this disease. It should not be used by pregnant women.
• Imatinib – this drug is used to prevent and stop the growth of the cancer cells. It is used to treat this medical condition, CML, stomach and digestive system tumors. This medicine should not be used by persons, who are pregnant, breast feeding, allergic to any component of this drug, by children, older adults. This medicine should not be used in combination with drugs such as: amphotericin B injectable, antineopastics, anti - thyroid agents, azathiopirine, chloramphenicol, colchicines.