Personal care treatments

Dermatofibrosarcoma Protuberans: causes, symptoms and treatments

Dermatofibrosarcoma protuberans (DFSP), also known as giant cell fibroblastoma, is a type of skin cancer that begins in the middle layer of the skin. In rare cases, it can spread to muscles, connective tissues or even bones.DFSP it’s rare and usually grows slowly. Although anyone can develop it, DFSP most of the time occurs among blacks. It also tends to develop faster in pregnant women.

Read on to learn more about the causes, symptoms, and treatments for dermatofibrosarcoma protuberance.

What causes protuberant dermatofibrosarcoma?

Liliya Rodnikova/Stocksy United

Although experts aren’t sure exactly what causes DFSP, they have observed some associated conditions:

  • It sometimes develops on severely injured skin or on scar tissue. Burn scars, scars from surgery, skin damaged by radiation treatments, and tattoos can be some of the affected areas.
  • Studies have also shown that a chromosomal translocation, or an unusual arrangement of chromosomes, could be linked to an occurrence of DFSP.

What are the symptoms of protuberant dermatofibrosarcoma?

DFSP is a slow-growing cancer, and you may not notice it for months or even years.

It can appear anywhere on the skin but usually forms on the shoulders and chest. In rare cases, DFSP can grow on the scalp or inside the mouth. Other locations may include:

  • abdomen
  • buttocks
  • return
  • arms
  • legs

In its early days, DFSP usually appears as an area of ​​hardened flesh-colored to reddish-brown skin. It often resembles a dermatofibroma, a common and harmless skin growth. On a newborn, it may look like a birthmark.

Eventually, the affected area develops into several purple to reddish-brown bumps or growths, which can reach several centimeters in diameter.

There is no pain or tenderness at first, but as it grows and stretches the top layer of skin, you may start to notice:

  • tenderness
  • hard bumps
  • open wounds
  • bleeding
  • cracks
  • pain

These changes happen very slowly at first. However, once more patches and bumps appear, the cancer begins to grow faster. If it reaches the lowest level of the skin, it can branch into the tissues like the roots of a tree, making treatment more difficult.

How do doctors diagnose protuberant dermatofibrosarcoma?

Doctors usually diagnose DFSP with a skin biopsy.

If a dermatologist or oncologist decides your cancer needs to be removed, they may order an MRI before surgery to determine how far it has spread.

What are the treatments for protuberant dermatofibrosarcoma?

More than likely, you will need to undergo surgery to remove the DFSP. A doctor will excise the tumor, removing the growth and some of the surrounding skin to catch any sections that have started to branch.

DFSP does not spread quickly but tends to reoccur in the same place, so it is essential to eliminate every patch of cancer.

The ideal method to remove the DFSP is usually Mohs surgery. A dermatologist surgeon will remove the tumor and a small portion of the surrounding skin, examine it under a microscope, and remove more skin until the margins are clear.

You may need reconstructive surgery to repair the scar if a deep excision is needed.

This can be difficult to remove all the cancer if it has spread to the lower levels of the skin and started to branch. If DFSP has spread or surgery isn’t an option, a dermatologist or oncologist may recommend additional treatments like radiation therapy or chemotherapy.

A chemotherapy drug called imatinib mesylate is available to treat DFSP. This drug only targets cancer-causing molecules, leaving surrounding cells mostly unharmed. However, the Food and Drug Administration has only approved it for certain situations:

  • when surgery to remove the cancer is not possible
  • when the cancer comes back after treatment
  • when the cancer has spread to other parts of the body
  • if you have certain DNA characteristics in your cells that make the medicine more likely to work

If chemotherapy shrinks the tumor enough, a doctor may be able to remove it surgically.

What is the outlook for people with protuberant dermatofibrosarcoma?

With treatment, Many people with DFSP live for many years. However, because DFSP often recurs in the same location, you may need lifelong follow-up appointments to check for it.

The 10-year survival rate for people with DFSP is 99.1%.

Although it is a slow growing cancer, it can spread to fat, muscle and even bone without diagnosis or treatment. Once it reaches the muscles or the bones, it becomes much more difficult to treat.

What are the potential complications of protuberant dermatofibrosarcoma?

The main complication of DFSP is its ability to metastasize, although this is rare. The lung is the most common site of metastasis.

Another possible complication is scarring resulting from surgical removal of the tumor.

DFSP has a high localized recurrence rate and usually recurs within 3 years.

What are the risk factors for protuberant dermatofibrosarcoma?

People 50 years and over have a high risk of DFSP recurring in the same location after removal.

If DFSP spreads to other parts of the body, the average life expectancy is about 2 years after diagnosis. Some risk factors that contribute to a higher risk of death from DFSP include:

  • to be a man
  • to be black
  • developing DFSP on the head, neck, arms, or legs

Frequently Asked Questions

Here are some other questions people frequently ask about DFSP. Julie Scott, DNP, ANP-BC, AOCNP, reviewed the responses.

How serious is dermatofibrosarcoma protuberans?

Although rare, DFSP can spread to other parts of the body and become difficult to treat. With prompt diagnosis and treatment, the outlook for people with DFSP is good.

Can protuberant dermatofibrosarcoma spread to the lungs?

Although DFSP does not usually spread to other parts of the body, the lungs are the The most common metastasis sites.

Yes, DFSP can come back after treatment. It tends to come back into the same place like the original tumour.

Dermatofibrosarcoma protuberans is a rare, slow-growing skin cancer. It usually begins as a flesh-colored or reddish-brown patch on the skin. Over time, it may progress to multiple purple to reddish-brown bumps.

The primary treatment for DFSP is surgical removal. Some people who can’t have surgery or whose cancer has spread may need additional treatments, including radiation therapy or chemotherapy.

It is important to keep all of your doctor’s appointments, even after surgery. This type of skin cancer often comes back in the same area, so it’s crucial to monitor your skin for new spots.

Contact your doctor if you notice any skin growths, changes in your skin, sores, or rough patches that won’t heal.