Ewing Sarcoma is a rare type of cancer that is most common in children and teens between the ages of 10 and 20. It is located in the bone or soft tissue around the bone, where in 87 percent of cases, the tumor starts growing in the bone, and in the remainder of cases, it begins in the cartilage or muscle around the bone. The most common places in which the tumors are found include the pelvis, the chest wall, and the legs.
Being a rare type of cancer, Ewing Sarcoma only affects about 200 children and young adults in the United States each year.
There are several types of Ewing Sarcoma, including Askin tumor, extraosseous Ewing sarcoma, and peripheral primitive neuroectodermal tumor (pPNET). Because these cancers share genetic sources, they are regarded as being related.
Sheba Medical Center in Israel offers comprehensive care to patients diagnosed with Ewing Sarcoma. As the largest hospital in the region, Sheba provides access to a multidisciplinary team of highly skilled medical professionals and pioneering treatments. Our approach encompasses all aspects of patient care, ensuring exceptional treatment for individuals from around the globe.
Patients with Ewing sarcoma receive specialized care from a team of experts who have received training at esteemed cancer centers worldwide. Recognizing that each case is unique, our experienced doctors collaborate to accurately diagnose, stage, and develop personalized treatment plans for every patient.
Ewing sarcoma is usually discovered after the patient complains of pain at the site of the tumor. The pelvis, legs, arms, and chest wall are the most common places for a Ewing sarcoma tumor to grow.
The symptoms of Ewing Sarcoma vary greatly depending on the size and location of the tumor, and in many cases, the symptoms are general, making the disease difficult to diagnose in the early stages.
Among common symptoms are:
- Constant or intermittent bone pain
- Swelling, tenderness or palpable lumps in the affected area
- Reduced overall physical activity and function, which could manifest in limping or stiffness if the sarcoma is the leg
- Loss of appetite, often accompanied by weight loss
- Broken bones for no known reason
These symptoms might be mistaken for signs of a more common illness like flu. In cases where the sarcoma has spread to other parts of the body (metastasized), there may be additional symptoms such as shortness of breath or chest pain if the lungs are involved, or bone pain in other parts of the body.
Ewing’s sarcoma is caused by a mutation in a cell’s DNA that causes it to multiply quickly and continue living when a healthy cell would normally die. The result is a mass (tumor) of abnormal cells that can invade and destroy healthy body tissue. In some cases, the abnormal cells spread throughout the body, most commonly to the bone marrow, lungs, kidneys, heart, adrenal glands, and other soft tissues, which results in metastatic Ewing’s sarcoma.
Ewing’s sarcoma accounts for about 1% of pediatric cancers, but is very rare in adults. It can occur at any time during childhood, but most commonly develops during puberty, when the bones are growing rapidly.
At present, there’s a limited understanding of Ewing Sarcoma risk factors. Unlike other forms of cancer, a genetic predisposition doesn’t seem to play a significant role in Ewing sarcoma cases. The disease is much more common among Caucasians compared to other ethnic groups and is more prevalent in males than females. Primarily, Ewing sarcoma affects children and young adults, with diagnoses in older adults being an extreme rarity.
The diagnosis of Ewing Sarcoma usually begins with a thorough physical examination and a review of the patient’s medical history. If the symptoms suggest Ewing Sarcoma, further testing such as imaging studies including X-rays, CT scans, MRIs, ultrasound, bone scans or PET scans may be ordered to visualize the tumor and check for metastasis.
A definitive diagnosis is usually made by a biopsy, where a sample of the suspected tumor tissue is removed and examined under a microscope by a pathologist and determines whether or not it is cancerous. The tissue can also undergo genetic tests to identify the specific chromosomal translocation typically seen in Ewing Sarcoma.
In recent years, advancements in molecular biology have revolutionized the diagnosis and treatment of Ewing sarcoma. Sheba utilizes these cutting-edge tests to aid in disease diagnosis and guide customized drug therapies for each patient, tailored to their individual needs and the specific stage of the disease.
Once a Ewing sarcoma diagnosis is made, doctors try to figure out how advanced the cancer is by determining whether it has spread, and if so, how much. Sheba’s specialists will gather all the information from the diagnostic tests, and perform further testing if necessary.
Stages 1 through 4 are used to signify how advanced the cancer is, with stage 1 being the least advanced, and stage 4 indicating that the cancer has spread to other parts of the body.
Instead of the traditional staging system used for most cancers, Ewing Sarcoma staging is usually classified into four types:
- Localized Ewing Sarcoma: The cancer is confined to the primary site where it started, and it has not spread to other parts of the body.
- Regional Ewing Sarcoma: In this stage, the cancer has spread to the nearby regions, and the 5-year relative survival rate drops to 71%.
- Metastatic Ewing Sarcoma: The cancer has spread from the primary site to other parts of the body.
- Recurrent Ewing Sarcoma: The cancer hasn’t responded to treatment or has returned after an initial response to treatment.
Ewing sarcoma treatment options depend on the type, size, and location of the tumor, and can include surgery, radiation therapy, chemotherapy, targeted drug treatment, immunotherapy, and bone marrow transplantation.
Based on the factors above, Sheba’s team of oncologists, hematologists, hematopathologists, and radiation oncologists will build a holistic personalized treatment for every patient, accounting for the physical and emotional aspects of the patient’s well-being.
Treatment for Ewing sarcoma typically involves a combination of chemotherapy, surgery, and radiation therapy.
Find out more information about the cutting-edge treatment options available for Ewing sarcoma at Sheba Medical Center.
Prognosis and Survival Rate
The prognosis for Ewing Sarcoma varies significantly depending on several factors, particularly the stage at which the disease is diagnosed and treatment begins.
For localized disease, where the cancer hasn’t spread beyond its origin, the prognosis is generally more favorable, with approximately 82% of patients living at least 5 years following diagnosis. In regional Ewing sarcoma, where the cancer has spread outside the bone (or other area) and into nearby structures, or it has reached nearby lymph nodes, the prognosis is approximately 71%. However, in cases where the sarcoma has metastasized to other parts of the body at the time of diagnosis, the prognosis is typically poorer, with roughly 15-39% of patients surviving at least 5 years.
It’s crucial to remember that these statistics are averages, and individual prognosis can vary based on factors like age, overall health, response to treatment, and advancements in therapeutic options. Factors other than the stage of the cancer can also affect survival rates.
Factors that have been linked with a better prognosis include:
- Smaller tumor size
- Main tumor is on an arm or leg (as opposed to chest wall or pelvis)
- Normal blood LDH level
- Good tumor response to chemotherapy
- Age younger than 10 years
Sheba's Ewing Sarcoma Experts
Being a relatively rare malignancy, quality treatment for Ewing Sarcoma can be hard to find. Therefore, it is critical to choose a medical center staffed with top tier cancer specialists and medical experts with experience in a wide range of disciplines for treatment.
At Sheba, we prioritize a holistic approach to healthcare, placing the patient and their family’s well-being at the forefront. We understand that cancer treatment impacts various aspects of one’s life, and we are dedicated to providing the best possible care.
Our Hemato-Oncology Division is dedicated to applying precision medicine for bone and soft tissue sarcomas. As a rule, we strive to customize the most effective treatment program for each individual patient. Sheba’s compassionate team of Ewing Sarcoma experts will ensure you are well-informed at every step of the way to healing.
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Sheba Medical Center provides innovative, personalized medical care to patients from around the world. We are the largest, most comprehensive hospital in the Middle East and dedicated to providing advanced and compassionate medicine for everyone. We welcome all cases, including the rarest and the most challenging. Our medical teams collaborate to provide the best possible health outcomes. From your initial inquiry through the long-term follow-up care, we are here for you.
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