Have you ever noticed a small bump or discoloration on your skin? Sometimes we don't pay much attention to it, right? But sometimes even something as small as this can be something to be concerned about. Today, we're going to talk about a rare but important type of skin cancer. It's called Dermatofibrosarcoma Protuberans , or DFSP for short.
What is DFSP (Dermatofibrosarcoma Protuberans)?
Simply put, DFSP is a slow-growing, but somewhat rare, type of skin cancer. It starts in the middle layer of our skin, the dermis . You may also hear a doctor call it DFSP.
Because this is a slow-growing cancer, it rarely spreads to other parts of the body. However, it is not a good idea to ignore it. It is very important to get proper treatment and remove this tumor. Only then can the cancer stop coming back (recurrence) or spread to other parts of the body (metastatic cancer) be prevented. With proper treatment, a person with DFSP has a very good chance of recovery.
Is this DFSP just a tumor? Or is it cancer?
No, DFSP is not just a benign tumor. It is a malignant condition. To be precise, it is a type of soft tissue cancer, a type of soft tissue sarcoma . Sarcomas are cancers that form in places like muscle, fat, and skin. Sometimes, they can also affect the bones.
Can DFSP spread (metastasize) to other parts of the body?
DFSP is not usually a type of cancer that spreads very quickly throughout the body. However, very rarely, about one in 20 people with DFSP can have the cancer spread beyond where it first started. This is called metastatic cancer .
This is most likely to spread if it is left untreated, or if the cancer has spread deep into the skin, into fatty tissue and muscle.
Another thing is, although the exact cause is not known, if DFSP develops during pregnancy, it may become more severe.
Also, a small number of people with DFSP can develop a more aggressive, faster-spreading type called fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS) . Even if these DFSP-FS tumors are surgically removed, they are more likely to come back and can spread to other areas.
How common is this condition called DFSP?
DFSP is a very rare type of cancer. Worldwide, it affects only about four people per million people per year. DFSP accounts for between 1% and 6% of all soft tissue sarcomas.
This cancer is most common in adults between the ages of 20 and 50. However, young children can also develop this skin cancer. Some babies can have DFSP at birth. Research has shown that black people are slightly more likely to develop it than people of other races.
What are the main types of DFSPs?
Pathologists, doctors who examine body tissue under a microscope, can tell you exactly what type of DFSP you have by examining the cells. There are several main types:
- Bednar tumors (also known as pigmented DFSP): These types of cells contain a lot of melanin , the pigment that gives skin its color. This is why these tumors can appear in a variety of colors, including red, brown, blue, and purple. This type accounts for about 5% of DFSP diagnoses.
- Giant cell fibroblastoma: This type is characterized by the presence of giant cells. This type is also called juvenile DFSP because it occurs most often in children and young adults.
- Fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS): As mentioned earlier, this is a relatively aggressive, rapidly spreading type of soft tissue sarcoma.
- Myxoid DFSPs: These are made up of an abnormal type of connective tissue called myxoid stroma. This type of DFSP is very rare.
What causes DFSP?
Surprisingly, 9 out of 10 people who develop DFSP have a genetic mutation that causes the condition . This gene mutation occurs in our cells after we are born. This means that DFSP is not caused by a genetic defect inherited from our parents.
What are the risk factors for developing DFSP?
In addition to race, having any type of skin injury or scarring can increase the risk of developing DFSP. There are several ways that skin can be injured:
- Burns
- Undergoing radiation therapy
- Surgical incisions
- Tattoos
Imagine, you have ever had a severe burn and a large scar. Or, you have had radiation treatment for another disease. When the skin is damaged by things like this, there is a small chance that a condition like DFSP will develop in the future, although rarely. That is why we should always be aware of changes in the skin.
What are the symptoms of DFSP?
The early symptoms of DFSP are so subtle that we may not notice them or even see them. These tumors most often develop on the chest, back, shoulders, abdomen, or buttocks. They can also develop on the arms, legs, scalp, and inside the mouth.
At first, you may see a small bump on the skin . It is flat and painless. It may feel a little rough to the touch, and the color may change slightly.
In infants and young children, DFSP can look like a birthmark . These spots are usually about half an inch to two inches (1 to 5 centimeters) wide.
The symptoms of DFSP become apparent as the cancer grows. As the tumor grows, it pushes into the top layer of skin (epidermis). This causes hard lumps (nodules) to appear on the skin's surface . The word "protuberance" means "protruding."
You may see these symptoms in these bumps:
- It can crack or bleed easily.
- It is firmly attached to the skin (cannot be moved around).
- As you get older, your skin stretches.
- It feels hard to the touch, or like rubber.
- The color can be reddish-brown, purple, blue, or red.
- It may be a little tender when touched.
Are there other conditions that cause symptoms similar to DFSP?
Yes, there is a non-cancerous skin condition called cellular dermatofibroma . This can look very similar to DFSP, especially in its early stages. Cellular dermatofibromas are benign soft tissue tumors. They most commonly occur on the legs. They can sometimes itch and be painful. Most dermatofibromas do not require treatment. However, it is wise to not confuse the two, so it is wise to see a doctor if you notice anything new on your skin.
How is DFSP (Dermatofibrosarcoma Protuberans) diagnosed?
DFSP is diagnosed by dermatologists , who are doctors who have special training in skin cancer and skin diseases. Your doctor will first take a small sample of the skin lump or spot and send it to a lab for testing (a skin biopsy) . The entire lump may be removed and sent to a lab. Pathologists will then look at the tissue under a microscope to see if it contains cancer cells.
If the skin biopsy confirms that you have DFSP, you may then be asked to undergo an MRI scan . This will help determine the exact size of the tumor and how deep it has spread into the skin. This information is very important for planning treatment.
What are the treatments for DFSP (Dermatofibrosarcoma Protuberans)?
The main and best treatment for DFSP is to remove the tumor surgically. Dermatologists usually use a special procedure called Mohs surgery to remove DFSP tumors. Here's what happens during this surgery:
- First, a local anesthetic is injected into the treatment area, so you won't feel any pain.
- Then, the doctor cuts out and removes the cancerous tumor. But not only the tumor, but also a small area of healthy skin around it. This is called the margin .
- Next, the edges (borders) of the removed tissue are examined under a microscope to see if any cancer cells remain.
- If there are still cancer cells at the edges, the doctor will remove some more tissue from the area where the surgery was performed again.
- In this way, tissue removal continues until no cancer cells are found at the margins.
- After the cancer is completely removed, reconstructive surgery is performed if necessary.
Are there other treatments for DFSP without surgery?
Even if DFSP tumors are surgically removed, they can sometimes come back. After a typical wide excision, there is a 20% to 30% chance that they will come back within three years. After Mohs surgery, this risk is reduced to 4% to 5%. However, it is important to remain vigilant, as tumors can recur even 10 years or more later.
Sometimes, if the cancer has spread to other parts of the body (metastatic DFSP), or if the tumor is too large or too localized to be removed surgically, doctors use a drug called Imatinib . This drug can also shrink the tumor, making it easier to remove surgically.
Also, if surgery cannot completely remove the cancer, or if surgery is not possible at all, radiation therapy may be used.
What is the outlook for someone with DFSP?
This is really comforting news. Since DFSP is a slow-growing, indolent cancer, with proper treatment, more than 99% of people will survive for 10 years or more. So, the most important thing is not to panic and follow your doctor's advice.
What can I do to improve my outlook?
There are several things you can do to improve your outlook:
- Do regular skin self-exams. Look for any new changes, spots, or lumps on your skin. If you notice anything, tell your doctor right away.
- After treatment, see your doctor for skin checkups at least every three to six months for the first three years. Then go for checkups once a year, or as directed by your doctor.
- Minimize sun exposure. UV rays from the sun increase the risk of other skin cancers. So, wear sunscreen when you go out in the sun, wear protective clothing , and limit your time outdoors during the hottest part of the day.
When should I see a doctor?
If you notice any new changes in your skin, especially if you have any of the following, be sure to see a doctor:
- New, persistent bumps or spots, or new skin growths.
- Changes in warts, birthmarks, old scars, or tattoos.
- If the bumps on the skin bleed easily.
Remember, skin changes can often be harmless. However, some changes can be a sign of skin cancer. So, if you have any doubts, it's best to see a doctor to get it sorted out.
What should I ask my doctor?
If you are diagnosed with DFSP, you may want to ask your doctor questions like:
- Why did I get this skin cancer?
- Am I at risk of developing other types of skin cancer?
- What is the best treatment for me?
- Is there a risk of this cancer spreading to other parts of the body?
- How often should I come for cancer screenings?
Knowing the answers to these questions will help you gain a better understanding of the condition and can help you clarify your treatment and future plans.
Finally, things to remember
Although DFSP is a rare skin cancer, it is very unlikely to spread to other parts of the body. However, surgical treatment is essential to stop the growth. Early detection and proper treatment can greatly improve your outlook. So, be aware of your skin and see a doctor if you notice anything unusual. You are not alone, and there are doctors who can help you on this journey.
` DFSP, skin cancer, skin cancer, skin lumps, Mohs surgery, cancer symptoms, skin diseases
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