What is Spindle Cell Sarcoma? Let's talk about it simply!

Have you ever been worried about something strange, like pain or swelling in your bones or soft areas like your muscles? Sometimes these can be normal. But, rarely, these symptoms can be a sign of something serious. So, today we are going to talk about a type of cancer that is a little rare, but important to know about. That is called Spindle Cell Sarcoma.

What exactly is Spindle Cell Sarcoma?

Simply put, Spindle Cell Sarcoma is a rare type of cancer that develops in the bones and soft tissues of our body. Did you know that 'sarcoma' refers to abnormal cell masses, or tumors, that develop in those bones and the tissues that connect to them?

Now, the special thing about Spindle Cell Sarcoma is that when you look at those abnormal cells under a microscope, they look like a 'spindle'. A spindle is a shape like a candle, with the sides thin and the middle a little thicker. That's why it got its name.

Because of advanced lab tests, doctors have been able to identify more than 75 types of sarcoma. Spindle cell sarcoma is one such subtype. In fact, the term 'spindle cell sarcoma' is used to describe sarcomas that cannot be accurately classified by certain lab tests, such as stains or genetic characteristics. So, it's a bit of a broad term.

Where do these tumors most commonly occur?

Spindle Cell Sarcoma most often develops in the long bones of our body. For example:

• Your thigh bone (`femur`)
• Shin bone (tibia)
• Upper arm bone (humerus)

However, it's not just these places. Some people have spindle cell sarcomas in the pelvis, head and neck, and other parts of the body.

What are the main types of Spindle Cell Sarcoma?

Now, look, all sarcomas start in soft tissue. That is, in things like fat, muscle, blood vessels, nerves, and the tissue around bones. Doctors further classify spindle cell sarcomas based on the type of cell in which the cancer starts.

By understanding the cell type and its characteristics in this way, doctors can assess how likely the cancer is to spread. This also helps them identify the best treatments.

Although 'spindle cell sarcoma' is a broad term, there are several main types:

• Undifferentiated Pleomorphic Sarcoma:As the name suggests, the cells in this type of cancer are 'undifferentiated'. This means that it is not clear what type of cell the cancer started from. The diseased cells in the tumor are 'pleomorphic', meaning they come in a variety of shapes and sizes. In other words, a tumor is a collection of cells from different types.
• Fibrosarcoma: These arise from cells called fibroblasts. Fibroblasts are the main building blocks of connective tissue in our bodies.
• Leiomyosarcoma: These start in smooth muscle cells. These are different from the cells in skeletal muscle tissue, which we use to move our bodies and get energy. Smooth muscle cells are found in our internal organs, body cavities, and some bones as soft tissue.

How common is this condition?

Like other types of sarcoma, spindle cell sarcoma is very rare . In fact, it accounts for less than 10% of all bone and soft tissue cancers. Keep in mind, it's so rare that your doctor may not suspect this type of cancer at first. You may have to go to several doctor's appointments, see several specialists, and have several tests before you receive a diagnosis.

Spindle cell sarcoma can develop in anyone. However, it is most common in people over the age of 40. People who develop the previously mentioned undifferentiated pleomorphic sarcoma are usually between the ages of 60 and 70.

What are the symptoms of this?

The symptoms of spindle cell sarcoma can vary. They depend on the size of the tumor, the number of tumors, and which bones (or other parts of the body) the cancer cells are in. Here are some symptoms you should especially look out for :

• Bone pain: This pain can be constant or come and go. It can feel like someone is stabbing you from the inside.
• A bone breaking at a point weakened by cancer: This is called a 'pathological fracture.' A bone can break even with a minor fall.
• Tenderness or swelling when pressed on the area where the tumor is: There may even be a tumor that is large enough to be held in the hand.
• Inability to move a joint close to the tumor properly: Imagine if the knee is close, it would be difficult to bend or extend the knee.
• Fatigue: This is not just normal fatigue. It is an extreme tiredness that does not go away no matter how much sleep or rest you get.
• Malaise: Feeling unwell , without any apparent reason, as if one has no energy or interest in anything.

What causes spindle cell sarcoma?

Like all cancers, spindle cell sarcoma begins when healthy cells become malignant. These malignant cells grow out of control, forming tumors. These tumors can damage surrounding healthy tissue. What's more dangerous is that these cancer cells can break away from the original tumor and spread throughout the body, damaging other tissues and organs. This is what we call metastasis, or metastatic cancer.

But, here's something to know. Normally, spindle cells (when they grow properly) are actually a good thing. Spindle cells form where tissue has been damaged. So, having spindle cells is a sign that your wound is healing. These cells disappear when the wound heals.

However, in spindle cell sarcoma, these spindle cells continue to grow and multiply. Scientists have not yet found a single specific reason why these cells become so malignant.

However, researchers have found that certain genetic mutations can increase a person's risk of developing this cancer. A 'mutation' is a mistake in our genes, the system that tells our cells how to function. In the case of spindle cell sarcoma, these mutations are often hereditary, meaning they can run in families.

What are the risk factors that increase the risk of developing this cancer?

There are several factors that can increase the risk of developing spindle cell sarcoma. Let's take a look at what they are:

• Bone Infarction: This is when bone tissue dies because it doesn't get enough blood. As you know, bone tissue needs blood to survive, which is what oxygen does.
• Fibrous Dysplasia: In this condition, healthy bone tissue is replaced by fibrous bone-like tissue. This increases the risk of fractures and (very rarely) can lead to the development of fibrosarcomas.
• Osteomyelitis: This is an infection that affects the spongy tissue inside some bones, called the bone marrow. This can happen if bacteria or other germs travel from your bloodstream to the bone and infect the cells there.
• Paget's disease of the bone: This is a long-term (chronic) condition. It causes bones to become weak and easily broken. This can increase the risk of developing bone cancer, including spindle cell sarcoma.
• Having previously received radiation therapy for cancer:Radiation therapy uses high-energy rays (often high-energy X-rays) to destroy cancer cells. Rarely, spindle cell sarcomas have developed in areas treated with radiation. When this happens, the sarcoma usually develops several years after treatment (seven to twenty years). But remember, less than 1% of people who receive radiation therapy develop this type of radiation-related cancer.

What are the complications of spindle cell sarcoma?

This cancer makes your bones more likely to break (fracture) at the site of the tumor. When a bone breaks, cancer cells from the tumor can travel to nearby areas.

However, there is still controversy about whether this type of bone fracture makes a difference to the overall prognosis (that is, the likely outcome) of the cancer, or whether the outcome is only related to the distant spread of the cancer (metastatic spread).

How is spindle cell sarcoma diagnosed?

Before your doctor can diagnose and stage your cancer, you will likely have a number of tests and procedures. Cancer staging allows doctors to classify the severity of the cancer based on the size of the tumor, its location, and whether it has spread. When staging bone cancer, they also look at whether the tumor is 'high-grade' or 'low-grade'. High-grade tumors have more abnormal -looking cells. They are more likely to grow and spread quickly.

Most spindle cell sarcomas are high-grade.

Imaging tests

Although imaging tests cannot tell the type of cancer, they can show the size and location of the tumor. They can also show whether the cancer has spread beyond the primary tumor.

• X-rays: X-rays can show tumors or other abnormalities in your bones. A chest X-ray can see if the cancer has spread to the lungs. Metastatic spindle cell sarcoma often spreads to the lungs first.
• MRI Scan: An MRI uses a large magnet, radio waves, and a computer to take pictures of things inside the body. Unlike X-rays, an MRI can show soft tissue in great detail. This is especially useful for doctors to identify soft tissue lumps that they suspect may be sarcomas.
• CT Scans: CT scans use X-rays and a computer to make three-dimensional (3D) pictures of bones and soft tissues. In the case of spindle cell sarcoma, CT scans can show whether the cancer has spread to soft tissues. They are also best for finding fractures or areas where the bones are weak.
• PET Scans: PET scans use a substance called a tracer to find cancer cells throughout the body. They can help find metastasized spindle cell sarcoma. You may also have a PET scan to see how your treatment is working. However, PET scans are not used for all types of sarcoma. Your doctor will decide if a PET scan is right for you.

Blood tests

There is no specific blood test that can detect spindle cell sarcoma. However, blood tests provide important information about your health, and it is important for your doctor to know that information. In general, blood tests can provide information about things like:

• Your blood cells
• Organ function
• Enzyme levels (enzymes are chemicals that help organs carry out important processes)

Biopsy

The best way to know for sure if a lump is cancerous is to have a biopsy. It is also the only way to know the type of cancer and its grade. During a biopsy, a doctor takes a sample of tissue from the lump and sends it to a lab to examine the cells.

Most people with spindle cell sarcoma have a needle biopsy. Doctors numb the tissue around the tumor and then use a small, hollow needle to take a sample. To help the doctor know exactly where in your body to insert the needle, you may also need an X-ray or CT scan.

How is spindle cell sarcoma treated?

The treatment you receive will depend on your health, the location, size, grade, and other factors. It also depends on whether you are receiving treatment for the first time or if the cancer has come back after treatment (recurrent).

Surgery

The goal of surgery is to remove the tumor while preserving as much of your bone as possible. If all traces of cancer in the primary tumor and secondary tumors can be removed, the cancer can be cured. However, once the cancer has spread beyond the original tumor, it is much more difficult to completely remove it.

Chemotherapy

Chemotherapy is a treatment that uses drugs to kill cancer cells throughout the body. You may be given chemotherapy before surgery to shrink the tumor and make it easier to remove. Or, you may be given chemotherapy to kill any remaining cancer cells after surgery. Chemotherapy is also used as 'palliative care'. Although it cannot cure cancer, chemotherapy can kill cancer cells to reduce symptoms such as bone pain.

Many people are given a combination of chemotherapy drugs to make the treatment more effective. Depending on whether you are receiving treatment for the first time, are not responding well to some of the chemo drugs, or if the cancer has come back after going into ``remission,'' you may be given different chemo drugs. ``Remission'' means that the cancer has either gotten better or gone completely after treatment.

Radiation Therapy

External Beam Radiation Therapy (EBRT) uses a machine to direct X-rays at the tumor to kill cancer cells. If you are unable to have surgery, radiation therapy may be given to destroy the tumor. Sometimes, a surgeon may not be able to reach the tumor surgically. It may be too deep in the tissue or too close to vital organs to be removed safely. In such cases, you may be given radiation therapy instead.

Like chemotherapy, radiation therapy can also reduce symptoms if the cancer cannot be cured.

What are the complications or side effects of the treatment?

It is common to experience side effects from chemotherapy and radiation therapy during cancer treatment. These may persist for some time after treatment has finished. It is important to ask your doctor about the possible side effects, depending on the treatment you are receiving.

When should I see my doctor?

Your doctor will tell you how often you need to come in for treatment, or to have tests to see if the cancer is still in ``remission.`` Many people with cancer who are in remission will need to go to the doctor more often in the early stages. As you stay cancer-free for longer, the number of follow-up appointments to check for recurrence will decrease.

What kind of follow-up treatment do I need?

Depending on how much bone the surgeon had to remove, you may need additional procedures. These procedures help to maintain the function of the bone. For example, you may need a bone graft. A bone graft involves replacing the removed tissue with tissue from another part of your body or from a donor. You may also need an artificial limb. You may need additional treatments to help you adjust to these changes.

What questions should I ask my doctor about spindle cell sarcoma?

Here are some important questions to ask your doctor:

• Where is the cancer?
• What is the stage/grade of cancer?
• How does that stage/grade affect my treatment plan?
• What treatment side effects should I expect?
• Can my cancer be cured?
• What are the goals of treatment?
• Can you connect me to palliative care services to help me cope with my cancer diagnosis?

Can spindle cell sarcoma be cured? How serious is it?

If all traces of the cancer can be removed, doctors can cure spindle cell sarcoma. Spindle cell sarcoma that has not spread beyond its original site is much easier to cure. However, treating sarcomas that have spread is very challenging . Metastatic Spindle Cell Sarcomas are rarely curable.

This type of cancer is often 'high-grade'. This means that it grows quickly and spreads quickly. After remission, it is more likely to come back, sometimes in the same place where the original tumor was, sometimes in a different place.

But remember, every cancer diagnosis is different. Your doctor can tell you the likely outcome of treatment (prognosis) based on the specific type of cancer you have, its stage, your overall health, your response to treatment, and a variety of other factors. Your doctor is the best person to advise you on how this diagnosis will affect you.

Things we should remember from this story (Take-Home Message)

Being diagnosed with Spindle Cell Sarcoma can be a stressful experience. The condition can make you feel tired and uncomfortable. Treatments are often intense, and you may face challenges such as getting used to a bone graft or an artificial limb.

Therefore, it is very important to have an honest and open conversation with your doctor from the beginning. This way, you can understand your diagnosis, the extent to which your cancer can be cured, what the goals of your treatment are, and what lifestyle changes you will need to make during treatment and recovery.

Spindle cell sarcoma is a serious, fast-growing cancer. However, there are treatments that can control its growth and, in some cases, cure it completely. Talk to your doctor about what you can expect. Never give up hope. Following proper medical advice and staying positive are very important in this fight.

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