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Ewing Sarcoma

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  3. Ewing Sarcoma

Overview of Ewing Sarcoma

Ewing sarcoma also known as (Sanooni astkhooni zakhira/ ثانوی استخوانی ذخیرہ) is a rare form of cancer that affects the tissues around the bones and appears as a solid tumor. Rarely, it affects the soft tissues in the chest, abdomen, limbs, or other locations.

Ewing sarcoma affects more in children and teenagers more, but it can occur at any age. It is more common in males than in females.

Ewing Sarcoma Prevalence

Ewing sarcoma is more prevalent between the ages of 10 and 20 or children less than 10. About a small percentage of people with Ewing's sarcoma are the ages of above 20 years.  According to JPMA, Ewing sarcoma cases affect 52% of females and 28% of males in Pakistan.

This cancer is the second leading cause of bone cancer in children. Its overall survival rate is about 70% of non-metastatic ES in developed countries.

Signs and Symptoms of Ewing Sarcoma

The symptoms of Ewing sarcoma may vary from person to person. Common symptoms include the following:

  • Feeling pain at the site of a tumor
  • Swelling or redness around the tumour area
  • Fever
  • Weight loss and decreased appetite
  • Fatigue
  • Paralysis or incontinence (for spinal region tumour)
  • Numbness, tingling or paralysis (if a tumour is in nerve area)

These symptoms of Ewing sarcoma may be similar to other medical conditions or problems.

Types of Ewing Sarcoma

The types of Ewing sarcoma consist of two major forms depending on the growth of cancer.

  • Benign Ewing Sarcoma- If the tumor is localized at one affected area of bones such as muscles or tendons, then it is called benign.
  • Malignant Ewing Sarcoma- If the tumor spreads to other areas of the body especially lungs or bone marrow then it is called malignant Ewing sarcoma

Causes of Ewing Sarcoma

Ewing sarcoma is caused by a mutated gene named EWSR1, due to which, cells in the affected area start to replicate to cause a lump over there. This lump is a tumor of Ewing sarcoma and it spreads to other areas of the body. The exact cause of how they produce is still not known but some risk factors can increase sarcoma.

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Risk Factors of Ewing Sarcoma

The following are the risk factors of Ewing sarcoma:

  • Age- The tumor of Ewing sarcoma forms at any age, but it is more likely to develop in children and teenagers.
  • Race- People with white tones affect more than darker tones.

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Prevention

There are no known ways to prevent wing sarcomas. The only risk factor of the disease is affected genetics so we can’t prevent the disease.

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Diagnosis

In addition to a complete medical history and physical examination, several diagnostic tests are performed for the determination of a tumour body. This helps the oncologists to decide the best treatment option. Diagnostic tests for Ewing sarcoma may include the following:

Multiple Imaging Tests

  • X-ray- The X-ray diagnostic test helps to produce scans of internal tissues, bones, and organs on film by passing through electromagnetic waves.

  • Radionuclide Bone Scan- In radionuclide bone scan test, the scan of bone images is obtained with a help of a small amount of radioactive dye. This dye is injected into the patient’s body and is absorbed by the bone tissue. These bone scans are used to determine any abnormalities and tumors in a specific area.

  • Magnetic Resonance Imaging (MRI)-The MRI test is performed for the determination of organs and structures within the body. Also, check the extent to the tumor that has spread to surrounding tissues.

  • Computed Tomography (CT or CAT) Scan-The CT Scan diagnostic imaging test is helpful for the detailed scan of bones, muscles, fat, and organs. Also, identify any abnormalities or growth of tumors within them.

  • Positron Emission Tomography (PET) Scan- In the PET Scan test, scans of the affected area is used to check any small tumor in it or also the extent of the tumor by injecting the radioactive-tagged glucose (sugar) into the blood. Affected tissues absorb more glucose than normal tissues (such as tumors) which become the marker of identification.

  • Blood Tests- The blood tests are used for blood chemistries and identify the affected gene EWSR1.

  • Biopsy of the tumor- In the biopsy test, a small number of tissue samples are removed (with a needle or during surgery) from the body for analysis. This is helpful for the determination of cancer or any abnormal cell growth present in the affected area.

  • Bone Marrow Aspiration Biopsy- During the bone marrow aspiration biopsy procedure, a small amount of bone marrow fluid and tissue from the affected area, mainly, the hip bone area is removed. To check the extent of cancer in the affected area whether it has spread to the bone marrow or not.

Treatment of Ewing Sarcoma | When to Consult a Doctor

The best approach to treat Ewing sarcoma treatment is chemotherapy. Along with it, other treatments are also briefly described below:

  • Chemotherapy- Some medications are used to kill cancer cells. They may be administered by mouth, or through a vein (IV). After that, these drugs shrink the tumor or remove cancer with other treatments including surgery or radiation therapy. 

After the surgery or radiation therapy, chemotherapy drugs are used to kill remaining cancer. If cancer spreads to other areas of the body, chemotherapy is also used along with advanced treatments.

  • Surgery- It is a surgical treatment to cut out the tumor (resection) from a small area of bone or an entire limb depending on the patient's condition. The implementation of surgery is based on the size and location of the tumor, as well as, tumor shrinkage after the chemotherapy.

  • Radiation Therapy- This procedure kills cancer cells or to prevent them from growing through high-energy X-rays or other types of radiation. Radiation is also used to treat the area of the body where surgery is difficult to conduct and would cause various unacceptable results such as loss of bowel or bladder function. Radiation therapy is an advanced technique to slow the growth of cancer and relieve pain. 

If individuals with Ewing sarcoma feel numbness, pain at the site of a tumor, swelling around the area of the tumor, fever, or sudden weight loss, consult an oncologist as soon as possible.

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