Dermatofibrosarcoma Protuberans, Causes, Diagnosis, Treatment, Prevention, Complications

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Dermatofibrosarcoma protuberans (DFSP) is a type of skin cancer that starts in the tissue in the middle layer of the skin (dermis). Initially, this cancer is characterized by bruises or wounds which then develop into lumps on the surface of the skin. DFSP usually appears on the trunk, arms, and legs.

Dermatofibrosarcoma protuberans (DFSP) is a very rare skin cancer. This cancer is experienced by 1 in 100,000–1,000,000 people per year. Although it can be experienced by anyone, DFSP most often occurs in men aged 20–50 years.

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Dermatofibrosarcoma Protuberans, Causes, Diagnosis, Treatment, Prevention, Complications

DFSP tends to grow slowly and rarely spreads to other parts of the body. Therefore, DFSP sufferers have a high chance of recovery. On the other hand, if left untreated, this cancer can grow into the layers of fat, muscle or bone, making it more difficult to treat.

Causes of Dermatofibrosarcoma Protuberans


Dermatofibrosarcoma protuberans (DFSP) occurs due to changes or mutations in the COL1A1 and PDGFB genes. The gene mutation causes cell production to become uncontrollable so that it accumulates in the skin and forms cancer cells. However, it is not known exactly what causes this gene mutation.

Risk factors for dermatofibrosarcoma protuberans

Although the exact cause is unknown, there are factors that can increase the risk of developing DFSP, including:

  • Male gender
  • 20–50 years old
  • Have an open wound from a burn or surgery
  • Have had radiation therapy (radiotherapy)

Symptoms of Dermatofibrosarcoma Protuberans


Dermatofibrosarcoma protuberans grows slowly so that the initial symptoms are sometimes overlooked. Early symptoms of DFSP include:

  • Thick skin
  • The surface of the skin feels rubbery or hard to the touch
  • The surface of the skin is brownish red
  • Lumps the size of pimples on the skin and are not painful
  • Skin feels rough


These early symptoms can develop over several months to several years, but in pregnant women they tend to develop more quickly.

In its development, the complaints experienced can be in the form of:

  • Lumps that enlarge and make the skin stretch even more
  • The skin at the lump site becomes cracked and bleeds
  • The skin color is blue or red in children, and reddish brown in adults


Most DFSP lumps grow on the shoulders and chest, but a small number can appear on the head or neck area. More severe symptoms may develop when the cancer becomes a larger lump.

When to see a doctor

Check with your doctor if you experience changes to the skin as mentioned above. However, please note, these complaints can also indicate other medical conditions. Therefore, early examination should be done to get an accurate diagnosis.

Diagnosis of Dermatofibrosarcoma Protuberans


To diagnose dermatofibrosarcoma protuberans, the doctor will conduct a question and answer session regarding the symptoms and complaints experienced by the patient, as well as his medical history. After that, the doctor will carry out a physical examination, especially on areas of the skin that are experiencing symptoms.

The doctor will also carry out several tests to confirm the diagnosis, namely:

Skin biopsy
A skin biopsy is done by taking a sample of skin tissue to be examined in a laboratory, to determine the presence of cancer cells.
Magnetic resonance imaging (MRI)
This examination is carried out using magnetic waves, to see whether or not the spread of cancer cells.
Genetic test
Although rarely performed, genetic tests can detect changes or mutations in genes that cause cells to grow uncontrollably.

Treatment of Dermatofibrosarcoma Protuberans


The main treatment for dermatofibrosarcoma protuberans (DFSP) is surgical removal of the tumor to remove cancer cells. Some of the surgical techniques that can be performed are:

Excision surgery
This procedure is done to remove cancer in the skin and healthy skin tissue that surrounds it. This procedure is done to ensure all cancer cells are removed.
    
Mohs surgery
This technique is done by removing cancer cells and a little healthy tissue around it. In Mohs surgery, less tissue is removed than in excision surgery. The doctor can also examine the edges of the cut tissue under a microscope, to make sure there are no cancer cells left.

Apart from surgery, other methods doctors can use to treat DFSP are:

Radiation therapy or radiotherapy

Radiotherapy is the use of special rays to kill cancer cells. This therapy is performed when the lining containing cancer cells cannot be removed completely by surgery.

Target therapy

Targeted therapy is performed on patients who cannot undergo surgery. In this therapy, the doctor will give drugs that specifically target cancer cells so that the cancer cells weaken, such as Imanitib.

Imanitib specifically attacks a mutated gene that causes cancer cells to die. Unlike chemotherapy in general, this drug can prevent serious damage to healthy cells. Even so, the doctor will check for mutations in the gene before giving imatinib.

In the case of DFSP that grows in the deep layers of the skin, the doctor will perform reconstructive surgery to repair the wound caused by the surgical removal of the cancer.

It should be noted that DSFP can recur. Therefore, doctors will make efforts to reduce the risk of DFSP recurrence, including:

  • Carrying out reconstructive surgery, to repair wounds arising from cancer removal surgery
  • Undergo radiation therapy (radiotherapy), to kill cancer cells
  • Asking the patient to undergo routine control every 6 months for 3 years, because DSFP generally recurs 3 years after being treated


Dermatofibrosarcoma Protuberans Complications


If left untreated, dermatofibrosarcoma protuberans (DSFP) can cause complications in the form of the spread of cancer cells to the surrounding tissue, muscle, fat or bone. In addition, cancer cells can also spread to other organs of the body, such as the lungs, brain, heart, or lymph nodes.

Prevention of Dermatofibrosarcoma Protuberans


Dermatofibrosarcoma protuberans (DFSP) is difficult to prevent, because the cause is unknown. However, because DFSP can be caused by open wounds or burns, preventive measures that can be taken include:

  • Keep skin clean
  • Do skin care if you have dry skin so it doesn't get irritated easily
  • Be careful when doing activities that use fire, such as cooking
  • Avoid exposure to sunlight that is too hot
  • Avoid exposure to chemicals

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