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Ewing sarcoma - Types, Causes, Symptoms, Diagnosis and Treatment

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Ewing sarcoma is a cancerous condition usually affecting children, especially in the first decade of life. Read this article to learn more about it.

Written by

Dr. Lochana .k

Medically reviewed by

Dr. Anuj Gupta

Published At March 17, 2022
Reviewed AtOctober 7, 2022

What Is Sarcoma?

Cancer conditions are named differently depending on the place of occurrence in the body. Cancer that can occur on the bone and muscles are known as sarcoma. If the sarcoma occurs on the soft tissues like nerves, blood vessels, muscles, and fat, then it is mentioned as soft tissue sarcoma. More than 70 types of sarcoma have been recorded, and each one has a different presentation and mode of treatment. Among them, angiosarcoma, chondrosarcoma, and liposarcoma are the common ones.

What Is Ewing Sarcoma?

Ewing sarcoma is a type of bone cancer that has a very rare occurrence. It is known to affect younger children and teenagers, especially in the first decade of life. The cancer is generally known to start from the long bones of the body and the pelvic bones. The classical site of occurrence is diaphysis of long bones. However, it has the capacity to start from any part of the bone. In cases where soft tissue involvement is seen, it begins in the abdomen, chest, and limbs. Both males and females are known to be affected by Ewing sarcoma. The other names of Ewing sarcoma are round cell sarcoma or PNET (peripheral neuroectodermal tumor). It is possible to cure the patients with Ewing sarcoma, but long-term monitoring is required.

What Are the Types of Ewing Sarcoma?

There are three types of Ewing sarcoma. They are:

  • Bone Tumor: The majority of the Ewing sarcoma is known to happen in the bones. This specifically includes bones of the thigh, ribs, pelvis, and shoulders.

  • Extra-Osseous Tumor: It is also known as a soft tissue tumor. It can involve all the muscles and cartilage. Older people are known to be affected when compared to other types. The commonest occurrence happens in the trunk region. This type is known to affect both genders equally.

  • Askin Tumor: It is a rare type of cancer occurring in the soft tissues. It is known to be a type of primitive neuroectodermal tumor that can occur in the chest region.

What Are the Causes of Ewing Sarcoma?

The actual cause for Ewing sarcoma is not identified. Some genetic mutations have contributed to the sarcoma condition. The prime functioning of the cell depends on the DNA (deoxyribonucleic acid) material. It sends a command to the other cells for regulating the functions. If there is any miscommunication due to the mutations, it might result in genetic problems and abnormal cell growth. These abnormal cells begin to spread and cause the destruction of healthy cells. In Ewing sarcoma, there is an alteration in the gene EWSR1. People who are having severe exposure to radiation are also known to be affected by Ewing sarcoma.

What Are the Symptoms of Ewing Sarcoma?

Patients who are having Ewing sarcoma might experience pain in the involved region. It might be seen along with stiffness in that area. An important characteristic of cancer is pain in the affected region that has been lasting for a prolonged duration of time. The other symptoms associated with Ewing sarcoma are:

  • Prolonged fever.

  • Weight loss.

  • Anemia.

  • The appearance of a lump on the skin that might feel warm on touch.

  • Bone pain that is known to increase by doing exercise. It is also known to aggravate during night time.

  • If the jaw is known to have a tumor, then the patient might experience numbness and pain in the facial region. Swelling of the jaw is most commonly seen in such cases.

  • If the tumor is occurring near the spine, then there will be a loss of control over the bladder, and the patient might suffer from paralysis.

  • Classically the presentation of Ewing sarcoma matches with the presentation of osteomyelitis (infection of bone).

What Are the Risk Factors of Ewing Sarcoma?

The risk factors associated with Ewing sarcoma are:

  • European race: People belonging to the European race are known to be affected more than other people. It is necessary to be aware of such conditions.

  • Age: Since children (less than 10 years of age) are susceptible to Ewing sarcoma, it is necessary for these people to be aware of it.

How Is Ewing Sarcoma Diagnosed?

Diagnosis requires the following procedures and tests:

  • Clinical Examination: The doctor will perform a clinical examination to check the involved site. The doctor will record the redness, swelling, and presence of lumps. Past medical history is an important one that has a lot of medical significance. You have to report to the doctor regarding the past usage of medications and surgical procedures.

  • X-rays: X-ray is the most preferred tool to identify the tumor area if it is suspected. You would require recommendations from your doctor to perform an X-ray. If the tumor is present on the long bones, X-ray imaging will show onion skin appearance (though considered classical of Ewing sarcoma but can also be seen in other tumors of bone like osteosarcoma).

  • MRI Scan (Magnetic Resonance Imaging Scan): It is a radiographic procedure that uses scanners to generate pictures of the body's organs.

  • Bone Scan: Bone scan is a type of imaging technique that helps in detecting several types of infections, injuries, and tumors of the bone.

  • Positron Emission Tomography: Positron emission tomography is an imaging technique that involves the usage of a radioactive substance to visualize the physiological processes and metabolic activities. It is rarely used for diagnosis of Ewing sarcoma.

  • Biopsy: The doctor will request you for a biopsy specimen. It is collected from the involved site and sent to the laboratory for analysis. The two types of biopsy procedures recommended for Ewing sarcoma are:

    • Needle Biopsy: In this procedure, a thin needle is inserted on the skin and guided to reach the tumor. Small pieces of tissues are removed from the tumor with the help of a needle.

    • Surgical Biopsy: In this procedure, the doctor makes an incision and takes tissue for diagnosis.

What Are the Treatment Options for Ewing Sarcoma?

The first-line treatment of Ewing sarcoma is chemotherapy.

  • Chemotherapy: It is a treatment that uses powerful medication to treat cancer cells. It is usually combined with other drugs depending on the health condition. The drugs have the capacity to shrink the tumor. Later, doctors can easily remove the tumor from the targeted site. It is done along with radiation therapy for some patients. When chemotherapy is given along with radiation therapy, the rate of prognosis is very good.

  • Radiation Therapy: It is a method that uses high energy radiation such as protons and X-rays. They help in destroying cancer cells. If Ewing sarcoma is located in a region that is not easily accessible by surgery, then radiation therapy is the best option. In advanced Ewing sarcoma cases, radiation therapy is known to alleviate pain and slow cancer growth.

  • Surgery: A surgery is planned after coordinating with the doctor. The surgical options involve removing the involved part of the bone. In some patients, additionally, a larger tissue is removed to prevent a recurrence. In rare cases, amputation of the limb is required.

  • Psychological Counseling: Patients with Ewing sarcoma are known to suffer severe depression. They would require proper psychological counseling to overcome their complicated health conditions.


Ewing sarcoma is a cancer of the bone that can be completely treated when detected at the early stages. However, long-term observation is needed. In this type of sarcoma, chemotherapy is first done to reduce the tumor size which is then followed by radiation therapy and surgery. Since Ewing sarcoma most commonly affects children below the age of ten years, prolonged pain at a particular site should not be neglected. Reach out to healthcare professionals if there is severe pain for long periods to rule out Ewing sarcoma.

For more help, contact icliniq.com.

Frequently Asked Questions


What Is the Ewing Sarcoma Survival Rate?

The American Cancer Society says the five-year survival rate for localized Ewing sarcoma is 70%. With metastatic disease, the five-year survival rate ranges from 15 % to 30%. It is yet to be determined how adults with Ewing sarcoma and children fare.


Can Ewing’s Sarcoma Be Treated?

Oncologists prescribe a five-drug combination that is administered alternatingly to children and adolescents for up to a year. Around 70 % of young patients with localized disease respond to this combination therapy.


Which Stage of Cancer Is Ewing’s Sarcoma?

Advanced cancer of the bones or surrounding soft tissue is known as Stage 4 Ewing sarcoma. Early diagnosis is best for treatment, but many factors affect a successful outcome. A rare form of cancer is Ewing sarcoma.


Which Child Is Most Likely to Develop an Ewing Sarcoma?

Most cases of Ewing sarcoma occur in adolescents during puberty or younger children. Race. Caucasians are more likely than Asian Americans or African Americans to develop Ewing sarcoma.


Can People Live a Full Life With Sarcoma?

Sarcoma has a 5-year survival rate of 65%. A localized account for approximately 60% of all sarcomas. 81% of people with localized sarcoma survive for five years.


Does Ewing’s Sarcoma Hurt?

Tenderness, pain, swelling, or stiffness in the bone or the surrounding tissue. Approximately 85% of children and young adults experience pain from Ewing sarcoma. Nighttime pain can be less intense and come and go.


What Is Ewing Sarcoma’s Final Stage?

The disease is at its most advanced stage in Stage 4 ES. It suggests that cancer has spread to distant organs and tissues in the body. Bone pain, fractures, fever, and weight loss are all possible signs. Chemotherapy, surgery, and radiation therapy might be options for stage 4 ES.


Where Does the Ewing Sarcoma Begin?

Ewing sarcoma, or sarcoma, is a rare form of bone or soft tissue cancer. Ewing sarcoma typically begins in the pelvis and leg bones but can also develop in any bone. It may occur less frequently in the chest, abdomen, limbs, or other soft tissues.


Is It Possible to Cure Ewing Sarcoma Without Surgery?

Radiation therapy is an option to treat Ewing sarcoma when chemotherapy fails or if surgery is ineffective or does not remove all tumor cells. External-beam radiation therapy, which uses a machine outside the body to deliver radiation, is the most common type of radiation treatment.


How Long Does Chemotherapy Last for Ewing Sarcoma?

Most patients will receive chemo for at least nine weeks before surgery or radiation, and they will also receive additional chemo afterward. Chemotherapy typically consists of 14 to 15 cycles, each of which can last anywhere from six months to close to a year, depending on the schedule.


How quickly does Ewing sarcoma grow?

Ewing's sarcoma and pPNET have no known causes. Tumor development typically occurs between the ages of 14 and 15 in young people because it appears to be linked to periods of rapid growth in life.


Is Ewing Sarcoma Dangerous to Life?

An aggressive cancer called Ewing sarcoma can potentially spread (metastasize) to the lungs, other bones, and bone marrow, which could result in life-threatening complications.


Is Ewing Sarcoma Inherited?

Ewing sarcoma appears to be caused by alterations in the chromosomes of a tumor cell, but the condition is not inherited. This indicates that it is not handed down from generation to generation. In addition, there is no known reason why genetic changes occur.


Is Ewing’s Sarcoma Preventable?

Age, gender, and race or ethnicity (the only known risk factors for Ewing tumors) cannot be changed. In addition, Ewing tumors have no known environmental or lifestyle causes, so there is currently no way to protect against them.


Who Is Most Likely to Develop Sarcoma?

Like most cancers, soft tissue sarcomas can occur at any age, but the risk increases. About 40% of people over 65 are diagnosed with soft tissue sarcomas or 40 out of every 100 cases. In addition, children and adolescents may develop sarcoma.
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Dr. Lochana .k
Dr. Lochana .k



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