Ewing (YOO-ing) sarcoma is a rare type of cancer that occurs in bones or in the soft tissue around the bones.

Ewing sarcoma most often begins in the leg bones and in the pelvis, but it can occur in any bone. Less often, it starts in the soft tissues of the chest, abdomen, limbs or other locations.

Ewing sarcoma is more common in children and teenagers, but it can occur at any age.

Major advancements in the treatment of Ewing sarcoma have helped to improve the outlook for people with this cancer. After completion of treatment, lifelong monitoring is recommended to watch for potential late effects of intense chemotherapy and radiation.

Symptoms

Signs and symptoms of Ewing sarcoma include:

• Pain, swelling or tenderness near the affected area
• Bone pain
• Unexplained tiredness
• Fever with no known cause
• Losing weight without trying

Risk factors

Risk factors for Ewing sarcoma include:

• Age. Ewing sarcoma can occur at any age, but it is more likely to occur in children and teenagers.
• Ancestry. Ewing sarcoma is more common in people of European ancestry. It's much less common in people of African and East Asian ancestry.

Diagnosis

• Imaging tests
• Removing a sample of cells for testing (biopsy)
• Testing the cancer cells for gene mutations

Treatment

Ewing sarcoma treatment usually begins with chemotherapy. Surgery to remove the cancer usually follows. Other treatments, including radiation therapy, might be used in certain situations.

Chemotherapy

Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy treatment usually combines two or more drugs that can be administered as an infusion into a vein (IV), in pill form, or through both methods.

Treatment for Ewing sarcoma usually begins with chemotherapy. The drugs may shrink the tumor and make it easier to remove the cancer with surgery or target with radiation therapy.

After surgery or radiation therapy, chemotherapy treatments might continue in order to kill any cancer cells that might remain.

For advanced cancer that spreads to other areas of the body, chemotherapy might help relieve pain and slow the growth of the cancer.

Surgery

The goal of surgery is to remove all of the cancer cells. Surgery for Ewing sarcoma may involve removing a small portion of bone or removing an entire limb. Whether surgeons can remove all of the cancer without removing the entire limb depends on several factors, such as the size and location of the tumor and whether it shrinks after chemotherapy.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells.

During radiation therapy, the beams of energy are delivered from a machine that moves around you as you lie on a table. The beams are carefully directed to the area of the Ewing sarcoma in order to reduce the risk of damage to surrounding healthy cells.

Radiation therapy might be recommended after surgery to kill any cancer cells that remain. It can also be used instead of surgery if the Ewing sarcoma is located in a part of the body where surgery is not possible or would result in unacceptable functional outcomes (such as loss of bowel or bladder function).

For advanced Ewing sarcomas, radiation therapy can slow the growth of the cancer and help relieve pain.

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