What do you think when you see a new lump or tumor on your body, especially on your leg or arm? Most of the time, we think it's normal and ignore it. But sometimes, even something as small as that can be something we need to pay attention to. Today, we're going to talk about a very rare cancer that can appear as a lump. This is called fibrosarcoma.
Simply put, what is fibrosarcoma?
Fibrosarcoma is a rare type of cancer that occurs in soft tissues. In medicine, we call this sarcoma. Now you may be wondering what soft tissue is. It's very simple. It's the tissue that connects and holds together different parts of our body. To be precise, things like tendons and ligaments that connect things like bones and muscles in our body belong to these connective tissues.
Fibrosarcoma cancer often develops in the soft tissues deep within the body. It is most common in the legs (around the hip or thigh bone), upper arms, knees, or trunk. It can also rarely develop in the head or neck. Sometimes, this cancer can also develop within the bones or in the fibrous connective tissue that covers the bones.
If you or your child receives this diagnosis, what happens next depends on many factors. Your age, the size of the cancer, and where it is located will all play a role. Your doctor will explain everything based on your diagnosis.
There are two main types of this.
Fibrosarcoma can be divided into two main types. These two types are very different from each other.
| Cancer type | Description |
|---|---|
| Infantile/Congenital Fibrosarcoma | This type is usually seen at birth or shortly after birth. Although this tumor grows rapidly, it is very unlikely to spread to other parts of the body. Therefore, it is a condition that can be completely cured . |
| Adult-type Fibrosarcoma | This type is most commonly seen in adults between the ages of 20 and 60. However, it can sometimes develop in older children and young adults. Unlike the infantile type, it is usually more aggressive and can be more difficult to treat. |
Fibrosarcoma is a very rare condition. It is rare in both adults and children. So don't be afraid that every lump that develops on the body is cancer. But it is wise to show a doctor about any new lump that develops.
What are the symptoms of this disease?
It usually takes a while for symptoms of fibrosarcoma to appear. Because these tumors develop in soft tissues deep within the body, you may not notice anything until the tumor has grown large enough to press on a nearby nerve or blood vessel.
These are the symptoms that are commonly seen:
- A soft lump that is painless or slightly painful: This can occur on the legs, arms, or trunk.
- A tingling sensation or sharp, stabbing pain: Sometimes we say "pins and needles." This may be due to pressure on a nerve near the tumor.
- Abnormal swelling: This type of swelling can occur when the tumor presses on blood vessels.
These symptoms can be similar to those of other, less serious conditions, so only a doctor can tell for sure whether these changes are due to fibrosarcoma or another common, non-cancerous (benign) condition.
What are the causes and risk factors for fibrosarcoma?
Researchers are not yet sure what exactly causes fibrosarcoma. However, it is believed that genetic mutations in cells play a role. Simply put, changes occur in the DNA in our cells. These changes cause cells to divide rapidly and uncontrollably, forming cancerous tumors.
A common genetic mutation has been identified in childhood fibrosarcoma, in particular. About 90% of these are related to problems in the NTRK gene family. When these genes do not work properly, cancerous tumors can develop.
Factors that increase risk
Researchers have found that certain hereditary conditions may increase the risk of developing fibrosarcoma.
- Familial adenomatous polyposis
- Li-Fraumeni syndrome
- Neurofibromatosis type 1 `(Neurofibromatosis type 1)`
- Nevoid basal cell carcinoma syndrome
- Retinoblastoma
- Tuberous sclerosis
- Werner syndrome `(Werner syndrome)`
In addition, some other medical conditions may be associated with this:
- Bone infarction (death of bone cells due to decreased blood flow to the bones)
- Chronic bone infection (Chronic osteomyelitis)
- Fibrous dysplasia
- Paget's disease of the bone
There are also risk factors related to the environment and your past medical history:
- Having previously received radiation therapy to the area where the cancer is located.
- Severe burns where the cancer is located.
- Exposure to certain chemicals, such as thorium dioxide, vinyl chloride, or arsenic.
- Exposure to metal parts (e.g. chromium, cobalt, nickel) inserted into the body through surgery.
How does a doctor diagnose this? (Diagnosis)
Doctors perform several tests to diagnose fibrosarcoma. The information from these tests determines the stage and grade of the cancer.
- MRI scan (Magnetic Resonance Imaging): This is the most common imaging test used to diagnose fibrosarcoma. It can clearly show the size and location of the tumor, and whether it is affecting blood vessels or nerves.
- CT scan (Computed Tomography scan): This uses a series of X-rays and a computer to create three-dimensional (3D) images of your soft tissues and bones.
- Biopsy: This involves removing a small piece of tissue from the tumor (core needle biopsy) or the entire tumor (excisional biopsy) for examination. A pathologist examines the tissue in a laboratory to see if it contains cancer cells.
- Immunohistochemistry (IHC): This is a special laboratory test that uses proteins called antibodies to confirm whether the tumor is fibrosarcoma or another type of soft tissue cancer.
How are the stages of cancer determined?
Doctors classify cancer into stages to determine how far it has spread and how serious it is. This takes into account the size of the tumor, its location, and whether it has spread. They also look at the "grade" of the cancer. This is how abnormal the cancer cells look when viewed under a microscope. Cells that look abnormal are considered "high-grade" cancers, which can be more aggressive.
| Stage | Description |
|---|---|
| Phase I | These are low-grade fibrosarcomas. If the tumor is larger than 5 centimeters, it is considered a more advanced stage. |
| Phase II | Mid-grade or high-grade fibrosarcoma. It is more advanced if the tumor is larger than 5 centimeters. |
| Stage III | High-grade fibrosarcoma. The tumor is larger than 5 centimeters and may have spread to nearby lymph nodes. |
| Stage IV | Fibrosarcoma of any grade or size has spread (metastasized) to distant organs or tissues in the body. |
Your doctor will explain to you what the outcome will be after treatment, depending on the stage of your cancer.
What are the treatments for this?
Treatment for fibrosarcoma depends on many factors, including your overall health, your preferences, and the type of cancer.
Treatment for adult-type fibrosarcoma
- Surgery: In this procedure, doctors remove the cancerous tumor and a small amount of healthy tissue around it to make sure that no cancer cells are left.
- Radiation Therapy: Radiation therapy may be given before surgery to shrink the tumor or to destroy any remaining cancer cells after surgery.
- Chemotherapy: Although some doctors prescribe this treatment, it is not very effective for adult-onset fibrosarcoma. Most of the time, this type of cancer does not respond well to chemotherapy.
Treatment for infantile fibrosarcoma
- Surgery: In children, surgical removal of the tumor can often completely cure the disease.
- Radiation and/or chemotherapy: These treatments are used to shrink tumors before surgery or to destroy remaining cells afterward. Unlike the adult form, most childhood fibrosarcomas respond very well to chemotherapy.
- Targeted Therapy: This treatment can stop cells from dividing rapidly due to problems with the `NTRK` gene. This can shrink tumors and make surgery easier.
How is the condition after treatment? (Prognosis)
The outcomes for infantile fibrosarcoma are very good. Most of these can be completely cured with a combination of surgery, radiation, chemotherapy, or targeted therapy. The 10-year survival rate is about 90%. If surgery can successfully remove all traces of the cancer, that figure approaches 100%.
Adult-onset fibrosarcoma is more complicated. These tumors are often diagnosed after they have grown large enough to affect nerves or blood flow. As with any cancer, early detection leads to better outcomes. Once the cancer has advanced, it becomes more difficult to treat. About half of patients may develop the disease again after treatment.
If you have this condition, how do you take care of yourself?
Many people feel helpless when they are diagnosed with cancer. One of the best ways to deal with these feelings is to take care of yourself during and after treatment. Here are some things you can do:
- Understand your follow-up plan: Ask your doctor what to expect during recovery and at follow-up appointments.
- Manage Anxiety: Regular check-ups after treatment are important for early detection of cancer recurrence. But it can also cause anxiety. Talk to a counselor about it.
- Ask for help: You will definitely need help during your treatment. Your loved ones may be waiting to help you. Tell them how you can help.
- Focus on good health habits: Eating nutritious foods, getting enough exercise, and sleeping well are very important.
- Rest: Cancer and cancer treatments can make you feel very tired. Plan to get as much rest as possible during treatment.
- Fight stress: Things like meditation, relaxation exercises, and deep breathing exercises can help reduce stress.
When do you need to see the doctor?
After treatment, you will usually see your doctor every three months for the first two years and then at slightly longer intervals for four to five years. If fibrosarcoma does recur, which usually happens within the first five years, this schedule will help detect any recurrence early.
You should contact your doctor any time you develop new lumps or new pain.
When do you need to go to the Emergency Treatment Unit (ETU)?
Many cancer treatments affect your immune system, which increases your risk of infection. Here are some symptoms that may require you to go to the Emergency Department (ETU) during treatment:
- A fever of 38.3 degrees Celsius (100.4 Fahrenheit) or higher.
- Feeling cold and shivering.
- Cough with mucus.
- Stomach pain.
- Persistent diarrhea.
- Persistent nausea and vomiting.
Take-Home Message
- Fibrosarcoma is a very rare type of cancer that forms in the body's connective tissues.
- There are two main types of this: the infantile type (often curable) and the adult type (more aggressive).
- Don't ignore a new lump, numbness, or unusual swelling in your body. See your doctor immediately.
- Treatment options (surgery, radiation, chemotherapy) depend on the type of cancer, its stage, and your health condition.
- Early detection of the disease is crucial for successful outcomes.
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