Description

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer. It begins in the cells of the connective tissue in the middle layer of skin (dermis).

Dermatofibrosarcoma protuberans may have the appearance of a grain or feel like a firm patch of skin at first. As it grows, lumps of tissue (protuberans is formed near the surface of the skin. This skin cancer often forms on the arms, legs, and trunk.

Dermatofibrosarcoma protuberans grows slowly and rarely spreads beyond the skin.

Symptoms

The symptoms of dermatofibrosarcoma protuberans include:

  • A lump or lumps in the skin that resemble pimples, scars or birth marks.
  • A lump or lumps in the skin that may feel hard to the touch, or rubber.
  • A patch of skin that feels firm to the touch.

The masses usually appear on the arms, legs or trunk. Rarely is formed in the head or neck.

When to see a doctor

Make an appointment with a doctor or other health care professional if they continue to symptoms that worry you.

Causes

The cause of dermatofibrosarcoma protuberans is often not known.

This skin cancer that occurs when cells in the skin to develop changes. The changes occur in the cells of the chromosomes. Chromosomes are threadlike structures inside cells that contain DNA and proteins. The changes in the chromosomes of the cause cells to make many more cells quickly. Cancer cells form a growth that starts below the skin and can push up to create a bundle over time. Other bumps can appear as well.

Risk factors

Risk factors for dermatofibrosarcoma protuberans include:

  • Age. This type of cancer can occur at any age. But it is more common in adults between the ages of 20 to 50 years.
  • Of the race. This type of cancer occurs more frequently in Black people than in people of other races.
  • Prior to the injury of the skin. People with skin that has been burned, treated with radiation or surgery scars may be more prone to developing this type of cancer.
  • The history of dermatofibrosarcoma protuberans. This type of cancer can return locally after surgical excision.

There is no way to prevent the dermatofibrosarcoma protuberans.

Diagnosis

Tests and procedures used to diagnose dermatofibrosarcoma protuberans include:

  • Examination of the skin. Your health care professional can inspect your skin for signs of skin cancer.
  • Skin biopsy. Your healthcare provider can remove a small amount of tissue for analysis. The tests in the lab to see if cancer cells are present.
  • Imaging tests. Sometimes imaging tests, such as magnetic resonance imaging, are needed to see the extent of the cancer and to help with the planning of the treatment.

Treatment

Dermatofibrosarcoma protuberans treatment usually involves surgery to remove the cancer. Other treatments may be used to kill cancer cells that may remain after surgery.

Treatment options may include:

  • The surgery to remove the cancer. Dermatofibrosarcoma protuberans tends to grow in a way that is not regular. This makes this cancer difficult to remove completely. Your health care professional may recommend a procedure to remove the cancer and some healthy tissue around it. This makes it more likely that all cancer cells are removed.
  • The Mohs surgery. Mohs surgery is a type of surgery that involves cutting of thin layers of cancer-containing skin until only cancer-free tissue is left. After each layer of skin is removed, it is examined for signs of cancer. The process continues until there are no signs of cancer. Mohs surgery can be useful for the treatment of major types of cancer.
  • The radiation therapy. Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill cancer cells. Your health care professional may recommend radiation therapy if all the cancer could not be removed during surgery.
  • The targeted therapy. The targeted therapy drugs attack specific chemicals present in the cancer cells. By blocking these chemicals, drugs targeted therapy that cause cancer cells to die. Some people with dermatofibrosarcoma protuberans has cancer of the cells that produce an excess of protein. A drug called imatinib (Gleevec) can be targeted to these cells and cause them to die. Your health care professional may recommend this treatment if surgery is not an option, or if the cancer returns after surgery.
  • Clinical trials. Clinical trials to test new treatments may be an option. Ask your health care professional if you are eligible to participate in a clinical trial.

Preparing for your appointment

Make an appointment with a doctor or other health care professional if you have any symptoms that worry you. If your healthcare provider believes you may have skin cancer, that person can refer you to a specialist. Often this is a doctor who specializes in the treatment of diseases of the skin, it is called a dermatologist.

Appointments can be brief, and being prepared can help. Here is some information that can help you prepare.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test. Make a list of:

  • Its symptoms, including those that do not seem to be related to the reason for your appointment.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your health care professional.

Have a friend or family member, if possible, to help you remember the information they give you.

For dermatofibrosarcoma protuberans, some basic questions to ask your health care professional include:

  • Do I have cancer?
  • If the lump or lumps are not cancerous, even I remove them?
  • I need more tests?
  • What are my treatment options?
  • What are the potential risks of these treatment options?
  • Do any of the treatments cure dermatofibrosarcoma protuberans?
  • I have a copy of my pathology report?
  • How much time can I take to consider my options for treatment?
  • Are there brochures or other printed material that I can take with me? What websites do you recommend?
  • What would happen if I chose not to have treatment?

Do not hesitate to ask other questions.

What to expect from your doctor

Your healthcare provider is likely to ask several questions, such as:

  • When did your symptoms begin?
  • The symptoms that has been happening all the time or come and go?
  • How severe are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Dermatofibrosarcoma protuberans