It's normal to feel a little anxious when you see a small lump or tumor somewhere on your body, right? At times like that, you think of a lot of things. "What is this? Is it dangerous?" Questions like these come to mind. Today we're going to talk about a very rare type of cancer that can appear as such lumps . That's called Epithelioid Sarcoma . Although the name may sound a bit strange, it's worth knowing about.
What is Epithelioid Sarcoma? To be precise…
Simply put, Epithelioid Sarcoma (ES) is a very rare type of cancer that belongs to a group of cancers called soft tissue sarcomas . “Soft tissue” refers to the things in our bodies like muscles, fatty tissue, blood vessels, and nerves. So this cancer develops in such places.
It often starts as a lump on your forearms, hands, fingers, lower legs, or soles of your feet . However, it can develop anywhere on your body. Sometimes, more than one lump can develop. Some people also develop ulcers on the skin where the lumps are.
The best part is, epithelioid sarcoma is a very slow-growing cancer . Just think, some people may have this type of cancer for months, even years, without showing any symptoms. That means it can grow inside your body without you even knowing it.
This is also called epithelial sarcoma. There are two main types of this: distal and proximal. Let's look at that too.
Are there any types of this? What are they?
Yes, as we said before, there are two main types of epithelioid sarcoma.
- Distal type: This is the most common type . “Distal” means far from the center of the body. This means that this type is most common in areas like your hands, feet, the front of your arms, and the lower part of your legs .
- Proximal type: This is the rarest type . “Proximal” means closer to the center of the body. So, this Proximal Epithelioid Sarcoma (PES) can develop in places like your chest, stomach, armpits, genitals (i.e. penis or vulva), or head and neck .
Usually, this proximal type is a little bit bigger than that distal type.Aggressive. That means it can spread a little faster and get worse. Because of that, it can be a little more challenging to treat.
How common is epithelioid sarcoma? Who is most likely to get it?
This is actually a very rare cancer. Just think, there are over 50 types of soft tissue sarcoma. All of them together, they account for about 1% of all cancers in adults. And then, this epithelioid sarcoma is, again, a very small number, about 1% of those soft tissue sarcomas. That means, it's really, really rare.
According to experts, even in a country like America, there are currently fewer than 1,000 people with this epithelioid sarcoma. So you can imagine how rare this is.
Usually,
- Distal type ES – the most common type – affects teenagers and young adults the most.
- Proximal type ES – the rare, slightly more severe type – is most commonly seen in older adults .
What are the symptoms? How do you recognize it?
In the early stages, epithelioid sarcoma may not cause any symptoms. This means you may not notice anything wrong with it. However, as the tumor grows, you may notice symptoms such as:
- A new lump or swelling appears. This can be small or large.
- Ulcerations form on the skin surface where the lump is located, similar to a small cut or wound.
- Pain is felt. But this only happens if the tumor is a little bigger and presses on the surrounding muscles or nerves. Not everyone experiences pain.
- It feels like a lump that has been there for a long time is getting bigger .
Important: If you notice a new lump or swelling like this anywhere on your body, or if you feel like an existing one is getting bigger, definitely see a doctor . It could be something simple, but it's best to get it checked out.
Why does this happen? What are the reasons?
To be honest, doctors still can't say for sure what exactly causes epithelioid sarcoma. However, research has found that it is associated with an abnormality in a gene called SMARCB1 . This gene is what tells our body to make proteins. This gene mutation usually occurs during life. That means it is not something you are born with, in most cases.
Like most soft tissue sarcomas, epithelioid sarcomas often develop without a clear cause . However, they are usually diagnosed after a previous cancer.The risk of developing this is slightly higher for people who have undergone radiation therapy.
Additionally, experts have found that people with certain family cancer syndromes may also be at slightly increased risk of developing epithelioid sarcoma. Some of these conditions include:
- Gardner syndrome
- Neurofibromatosis Type 1 and Type 2
- Retinoblastoma
- Li-Fraumeni syndrome
- Tuberous sclerosis complex
- Werner syndrome
- Gorlin syndrome
This is a bit complicated, but it's good to know. If you have this condition, talk to your doctor for further advice.
Does this disease spread throughout the body?
In most cases, epithelioid sarcoma does not spread (metastasize) to other places. That is, it stays where it started. However, sometimes, especially the more aggressive types like the proximal type, it can spread. If it does, it is most likely to go to your lymph nodes, lungs, and bones .
How do you recognize this? (Diagnosis)
A doctor will perform several tests to determine if you have epithelioid sarcoma. The main ones are:
- Physical examination : The doctor will examine you, checking things like the location of the lump and its size.
- Biopsy: This is the most important test . A very small piece of the lump is taken and examined under a microscope. Only then can we tell for sure if it is cancer, and if so, what type.
- Blood tests for cancer: Some cancer markers can be found in the blood.
- MRI scan (Magnetic Resonance Imaging – MRI): This can clearly see the location of the tumor, its size, and whether it has spread to surrounding tissues.
- CT scan (Computed Tomography – CT scans): This can also look at the tumor and see if the cancer has spread to other areas, such as the lungs.
- PET scan (Positron Emission Tomography – PET scans): This helps find areas in the body where cancer cells are active.
You may not need to do all of these tests. Your doctor will decide which tests are necessary based on your condition.
What is the treatment?
Your doctor will choose a treatment plan based on several factors, including the size of the tumor, its location, whether the cancer has spread to other areas, your body's ability to heal, and your preferences.
Surgery
The main and most common treatment for epithelioid sarcoma is to remove the tumor with surgery . If the cancer has spread to nearby lymph nodes, the surgeon may remove them as well.
Radiation Therapy
This is another common treatment for cancer. It uses high-energy X-rays to destroy cancer cells. Doctors may give this treatment to shrink a tumor before surgery. Or, it may be used to kill any remaining cancer cells after surgery.
Chemotherapy
If the cancer has spread to other parts of your body, your doctor may recommend chemotherapy . This involves giving you special drugs to kill cancer cells. These drugs are often given intravenously. But they can also be given as pills, a liquid, a cream applied to the skin, or an injection.
Targeted Drug Therapy
This is a relatively new treatment. It involves giving drugs that target only the genes and proteins that control how cancer cells grow and divide. This means that healthy cells are less likely to be harmed. There are several types of drugs used for this. Your doctor will create a specific treatment plan for you based on your condition. Tazemetostat is a type of drug used for epithelioid sarcoma. It helps treat cancer cells that cannot be completely removed by surgery.
Can this be prevented from happening?
The sad thing is, because epithelioid sarcoma usually develops for no apparent reason, we have no way to prevent it from developing . That means it's hard to say, "If you do this, it won't happen."
What happens if you develop epithelioid sarcoma? Can it be cured?
It's normal to feel scared when you hear this. However, epithelioid sarcoma is a treatable disease , especially if it's caught early . Your doctor will do some tests to see how big the tumor is and whether it has spread to other areas. Then, they'll create a treatment plan that's right for you.
There may be a team of doctors treating you. Among them are:
- Your primary care physician ( the doctor who usually sees you).
- Oncologists: These include orthopedic oncologists, oncology surgeons, and doctors who administer chemotherapy and radiation therapy.
- Dermatologists: For skin problems.
- Pathologists: Those who look at your blood and biopsy test reports and give you details.
- Therapists: People who help you deal with the stress and fears that come with an illness like this.
The only way to completely cure epithelioid sarcoma is to completely remove the tumor with surgery . However, it may not always be possible to completely remove it.
If a surgeon cannot completely remove the tumor, your doctor will often recommend a combination of treatments to control your condition. Because epithelioid sarcoma is a slow-growing cancer, many people with this disease can still live long, happy lives .
There are many research reports on the survival rate of this disease. However, because it is a very rare disease, the statistics vary greatly. According to some reports, the five-year survival rate ranges from 25% to 92%. It depends on many factors, such as the nature of the disease and the response to treatment.
When should I see a doctor?
If you notice an unusual lump or swelling anywhere on your body, don't ignore it. Be sure to see a doctor. People with epithelioid sarcoma most often develop lumps on the forearms, hands, fingers, lower legs, or soles of the feet. But remember, these tumors can develop anywhere on the body.
What questions should you ask the doctor?
If you or a loved one is diagnosed with epithelioid sarcoma, here are some questions you can ask your doctor:
- Where is the tumor?
- Do I only have one tumor? Or are there others?
- Has the cancer spread to other parts of the body?
- What treatment do you recommend?
- How long will my treatment take?
- How will I feel during treatment? (Things like side effects)
- Are there any places where I can learn more about this? (like trusted websites, books)
It's normal to feel scared, angry, and frustrated when you receive a diagnosis like this. Talk to your doctor about what to expect before, during, and after treatment. It can also be helpful to talk to a counselor or therapist. They can help you deal with the complex emotions you're going through at a time like this. If possible, join a support group with people who are going through the same thing as you. This can help you feel less alone.
Take-Home Message
- Epithelioid sarcoma is a very rare, aggressive soft tissue cancer .
- Often lumps in the limbsIt can appear as a rash, sometimes with skin lesions.
- Symptoms may not appear in the early stages.
- It is associated with a defect in a gene called SMARCB1 , but the exact cause is not yet known.
- Surgery is the main treatment . Radiation therapy, chemotherapy, and targeted drugs are also used.
- It is difficult to prevent , but if identified early, it can be treated .
- If you notice a new lump on your body, see a doctor immediately.
- Don't be afraid, don't panic, and follow proper medical advice . You are not alone, there are doctors and loved ones to help you.
Remember, this article is for general information only. If you have any concerns, it is best to consult a doctor.
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