Ewing’s Sarcoma: Symptoms, Causes, Diagnosis and Treatment

Overview

Ewing sarcoma is a rare bone or soft tissue cancer largely affecting children and teenagers but can also affect adults. It occurs due to mutation in the cells that create abnormal cells. Ewing sarcoma affects men more than women. 

The blog explains Ewing sarcoma, its causes, types, symptoms, causes, and various treatment options. 

What is Ewing Sarcoma?

As mentioned above, Ewing sarcoma is rare cancer affecting bone and soft tissues. Typically, it starts in the bones of the legs and pelvis, but it can occur in any bone. In rare cases, it may begin in the soft tissues of the chest, abdomen, limbs, and other body parts. It can develop at any age, but it mainly affects children and young adults. 

There is no possible way to prevent Ewing sarcoma. However, you can recover completely if diagnosed early.

What are the Different Types of Ewing Sarcoma?

Ewing sarcoma typically starts at your pelvis, followed by the femur or thigh bone. However, it can spread to other bones, bone marrow, and sometimes vital organs, including your lungs, heart, and kidneys. The different types of Ewing sarcoma are as follows:

1. Ewing sarcoma of bone : This is the most common type of tumor in the Ewing sarcoma family. In 192, Dr. James Ewing first described it as its appearance under the microscope differed from other common bone tumors, osteosarcoma. This type of sarcoma is known to respond to radiation therapy.
2. Extra-osseous Ewing Tumor : Cancer affects the soft tissues around your bones, including cartilage and muscles. 
3. Peripheral primitive neuroectodermal tumor (PPNET) : It is a rare childhood cancer. The tumors develop in the bone or soft tissue. The tumor shares several features with Ewing sarcoma of bone and extra-osseous Ewing tumor. However, PPNET, which begins in your chest wall, is known as an Askin tumor. 

What are the symptoms of Ewing Sarcoma?

Pain, stiffness, and swelling are the most common symptoms of Ewing sarcoma that often get confused with growing pain in children and sports injuries in adults. The other symptoms of Ewing sarcoma are:

• A visible lump that is tender and warm to touch
• Unexplained weight loss and fever
• Limping due to pain in the legs
• Easily broken bone without a cause
• Easily getting tired
• If the tumor is near the spine, causing paralysis, or loss of bladder control
• Loss of appetite
• Anemia

When to Seek Medical Advice?

Book an appointment with your physician if you notice any of the above symptoms.

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What are the Causes of Ewing Sarcoma?

The reason for developing Ewing sarcoma is not known. Experts believe that Ewing sarcoma starts when there is a change in your DNA cells. These cells hold instructions that instruct cells what to do. When there is a change in the cell, it is instructed to multiply and destroy healthy cells rapidly. It results in tumors of abnormal cells that may break away and spread to other body parts. 

The changes in your DNA most often affect the EWSR1 gene. If your doctor doubts that you are suffering from Ewing sarcoma, your cells are tested for the changes in this gene.

How is Ewing Sarcoma Diagnosed?

If your doctors suspect that you have Ewing sarcoma, they may prescribe the following tests: 

1. Physical exam: Most doctors begin with a physical exam. The doctors check for signs of lumps, redness, and swelling.  
2. X-rays: The x-ray images show your doctor any issues in your bone. If there is an abnormality, your doctor may suggest other imaging tests.
3. Bone scan: It shows if cancer cells are present in the bones. You will be intravenously injected with a radioactive dye that collects in the bone tumor during the procedure. It shows up in the bone scan.
4. MRI scan: This imagining test uses a strong magnetic field and radio waves to create detailed images of your body.
5. CAT or CT scan: Similar to an MRI scan, it shows detailed imaging of your body.
6. PET or Positron emission tomography scan
7. Biopsy: Your healthcare provider may remove a piece of the cancerous tissue to check and confirm the diagnosis. Needle biopsy and surgical biopsy are two types of biopsies. 
8. Gene mutations testing: Cancer cells that are removed through a biopsy are tested to check if there is a change in the DNA. Thus, confirming the diagnosis of Ewing sarcoma. Genetic testing also helps your doctor in assessing the aggressiveness of your cancer.

What are the Various Treatment Options for Ewing Sarcoma?

Your doctor determines your treatment options based on the size of your tumor, its severity, and overall health. The treatment options are as follows:

1. Chemotherapy: It is the first step of your treatment. In this, your doctor injects your with medicines to kill and prevent the cancer cells from growing. Your physician may combine chemotherapy with radiation and surgery. 
2. Surgery: Your doctor surgically removes the tumor and prevents it from spreading to other body parts. Sometimes, the doctor may amputate your limb if the tumor has spread exponentially. 
3. Radiation therapy: In this, your doctor uses high-energy beams to kill and prevent cancerous cells from rapidly spreading. This treatment option is usually suggested when the doctor cannot altogether remove the tumor surgically and your cancer does not respond to chemotherapy. 

You would need to continue meeting your team of doctors for follow-up checkups, as it may reoccur decades after the treatment. 

What are the Complications of Ewing Sarcoma?

The complications of Ewing sarcoma and its treatments include the following:

• Cancer may metastasize to other parts of your body. Thus, making it challenging to treat and recover from it. It often spreads to the lungs and other bones.
• The aggressive treatment options have substantial side effects – short and long-term. However, your doctor may be able to manage and monitor these side effects.

Conclusion

Getting a cancer diagnosis is challenging at any age. It is particularly difficult when it comes to children. However, it is vital to understand that as science and medicine advance, the prognosis for Ewing sarcoma is good if diagnosed and treated at the earliest. 

Frequently Asked Questions (FAQs)

What are the other names of Ewing sarcoma?

It is also called Ewing’s sarcoma and Ewing tumor. 

Who gets affected by Ewing sarcoma?

Young men are more prone to develop Ewing sarcoma than women. It typically occurs during puberty as young people’s bones rapidly grow.