Ewing’s Sarcoma - Early Signs, Risk Factors, Diagnosis, and Treatment Explained

Ewing's sarcoma is a rare type of cancer that typically develops in the bones or soft tissues of children and young adults. Because its symptoms—such as bone pain or swelling—can be mistaken for more common childhood ailments, understanding this condition is especially important. This comprehensive guide provides clear, compassionate, and easy-to-understand information about Ewing's sarcoma—covering its symptoms, how it is diagnosed, available treatment options, and what to expect throughout the journey. Our goal is to help you feel informed, supported, and empowered to face this challenge with confidence and hope. 

What Is Ewing's Sarcoma? 

Ewing's sarcoma is a very rare and aggressive type of cancer. It is a member of a group of tumors known as the "Ewing sarcoma family of tumors." The cancer begins as a growth of cells in the bones or in the soft tissues around the bones. While it can occur at any age, it is most commonly diagnosed in children and young adults, often in their teenage years. 

Ewing's sarcoma is the second most common type of primary bone cancer in children and young adults, after osteosarcoma. The most common locations for the tumor to form are the pelvis, ribs, and the long bones of the legs and arms. The good news is that with modern, intensive, and multidisciplinary treatment, the prognosis for most children and young adults with Ewing's sarcoma has improved dramatically over the past few decades. 

Types of Ewing's Sarcoma 

Ewing's sarcoma is classified into three main types, which are based on where the tumor originates. 

• Ewing's Sarcoma of the Bone: This is the most common type. It starts in the bone itself. 

• Extraosseous Ewing's Sarcoma: This type starts in the soft tissues outside of the bone, such as in the muscles or fat. 

• Peripheral Primitive Neuroectodermal Tumor (PPNET): This is a rare type of cancer that shares many characteristics with Ewing's sarcoma. 

The distinction between these types is important, but from a treatment perspective, they are all treated in a similar, aggressive way. 

What Are the Causes and Risk Factors for Ewing's Sarcoma? 

In the vast majority of cases, the exact cause of Ewing's sarcoma is unknown. It is not caused by a single factor, but rather a specific genetic change that happens during a person's lifetime. 

• Genetic Change: In over 95% of Ewing's sarcoma cases, there is a specific genetic mutation called a "chromosomal translocation." This means that a part of one chromosome has broken off and attached to another chromosome. This creates a "fusion gene" called EWSR1-FLI1, which is believed to be the driving force behind the development of the cancer. 

• Age: Ewing's sarcoma is most common in teenagers and young adults, with the peak incidence occurring in the second decade of life. 

• Race/Ethnicity: Ewing's sarcoma is much more common in people of European descent and is very rare in people of African or Asian descent. 

It is important to remember that this genetic change is not inherited. It occurs randomly and is not passed down through families. Parents should not blame themselves for their child's diagnosis. 

What Are the Symptoms of Ewing's Sarcoma? 

The symptoms of Ewing's sarcoma can be vague and are often mistaken for other, more common conditions like sports injuries or "growing pains." However, if you notice these symptoms, especially if they are persistent, it is important to see a doctor for a proper evaluation. 

Common Early Signs: 

• Persistent Pain: The most common symptom is a persistent pain in the affected bone or soft tissue. The pain may come and go at first, but it can become constant and may get worse at night or with activity. 

• A Lump or Swelling: A noticeable lump or swelling may appear in the affected area, which may feel warm and soft to the touch. 

• Unexplained Fever: A persistent, unexplained fever that comes and goes. 

• Unexplained Broken Bone (Fracture): In rare cases, a bone can become so weakened by the cancer that it breaks after a minor fall or injury. 

Advanced Symptoms: 

As the cancer progresses, other symptoms may appear: 

• Unexplained Weight Loss: Losing weight without trying. 

• Fatigue: Feeling unusually tired or a general lack of energy. 

• Breathing Problems: If the cancer has spread to the lungs, it can cause a persistent cough or shortness of breath. 

If your child has a persistent bone pain or a lump that doesn't go away, it is crucial to consult a doctor right away for an accurate diagnosis. 

How Is Ewing's Sarcoma Diagnosed? 

Diagnosing Ewing's sarcoma requires a series of specialized tests to confirm the presence of cancer, determine its type, and see if it has spread. The process often begins with a physical exam and a detailed discussion of your child's symptoms. 

Diagnostic Steps and Tests: 

1. Physical Exam and Medical History: Your child's doctor will ask about their symptoms and risk factors. They will also perform a physical exam to check for a lump, swelling, or pain. 

2. Imaging Scans: Imaging scans are crucial for seeing any abnormalities in the bone. 

• X-ray: An X-ray is often the first test used to find a bone tumor. 

• MRI (Magnetic Resonance Imaging) Scan: An MRI provides even more detailed images of the bone and soft tissues, which helps doctors determine the size and extent of the tumor. 

• CT (Computed Tomography) Scan: A CT scan provides detailed, cross-sectional images of the bone. A CT scan of the chest is also used to check if the cancer has spread to the lungs, which is the most common site of metastasis. 

• PET (Positron Emission Tomography) Scan: A PET scan can be very helpful as it can identify active cancer cells throughout the body. 

3. Biopsy (The Definitive Test): A biopsy is the only way to definitively diagnose Ewing's sarcoma. A small tissue sample from the suspicious area is removed and sent to a lab to be examined by a pathologist. The pathologist will use special tests to look for the specific genetic fusion gene to confirm the diagnosis. 

4. Bone Marrow Aspiration and Biopsy: A small sample of bone marrow is removed from the hip bone to check if the cancer has spread to the bone marrow. This is an important step for staging. 

It is highly recommended that the biopsy be performed at a specialized cancer center with a team experienced in Ewing's sarcoma care, as an improper biopsy can sometimes compromise future treatment. 

Staging and Grading of Ewing's Sarcoma 

Ewing's sarcoma is considered a high-grade and aggressive cancer. Therefore, it is not "graded" in the traditional sense. The key to treatment planning is "staging," which determines the extent of the cancer's spread. The most common staging system is a two-stage system. 

• Localized: The cancer is confined to the bone or soft tissue where it started and has not spread to distant parts of the body. 

• Metastatic: The cancer has spread to distant parts of the body, most commonly the lungs, other bones, or the bone marrow. 

It is important to remember that even if the cancer appears to be localized on scans, doctors will treat it as if some small, undetectable cancer cells (micrometastases) have already spread. This is why chemotherapy, which works throughout the entire body, is a crucial part of the treatment for all patients with Ewing's sarcoma. 

What Are the Treatment Options for Ewing's Sarcoma? 

The treatment plan for Ewing's sarcoma is highly personalized and depends on the stage, the child's age, and overall health. It is a multidisciplinary approach that combines several therapies. 

1. Medical Treatment (Chemotherapy, Targeted Therapy, Immunotherapy) 

• Chemotherapy: Chemotherapy is the primary treatment for Ewing's sarcoma. It is almost always given before surgery to shrink the tumor and after surgery to kill any remaining cancer cells. This combination of chemotherapy and a local treatment (surgery or radiation) has dramatically improved survival rates. 

• Targeted Therapy and Immunotherapy: Targeted therapies and immunotherapy are a promising area of research for Ewing's sarcoma. These drugs are being studied for their potential use in treating this aggressive cancer. 

2. Surgery 

Surgery is used to remove the tumor after a course of chemotherapy has shrunk it. The goal is to remove the entire tumor with a margin of healthy tissue around it. 

• Limb-Sparing Surgery: For tumors in the arms or legs, this is the most common approach. The surgeon removes the part of the bone with the tumor and replaces it with a metal implant (prosthesis) or a bone graft from another part of the body. This allows the child to keep their limb. 

• Amputation: In rare cases, if the tumor is very large, has invaded vital nerves or blood vessels, or cannot be fully removed with limb-sparing surgery, amputation may be necessary. Amputation is a last resort. 

3. Radiation Therapy 

Radiation therapy uses high-energy rays to kill cancer cells. It is a critical part of the treatment for Ewing's sarcoma, especially for tumors that are in a location where surgery is not possible, such as the pelvis or spine. It is also used in combination with chemotherapy to treat tumors that were not completely removed with surgery. 

4. Proton Therapy 

Proton therapy is a highly advanced form of radiation that uses proton beams instead of X-rays. Because protons can be precisely aimed to deliver a high dose of radiation directly to the tumor while sparing nearby healthy tissues and organs, it may be a good option for Ewing's sarcoma located near sensitive structures, such as the spine or pelvis. 

Prognosis and Survival Rates for Ewing's Sarcoma 

The prognosis (the likely outcome of the disease) for Ewing's sarcoma has improved dramatically in recent decades. The outlook depends on the tumor's size, its location, and whether it has spread. 

• Prognostic Factors: The most important factors affecting prognosis are whether the cancer is localized or metastatic at diagnosis, the tumor's size, and how well the tumor responds to chemotherapy. 

• Survival Rates: The 5-year survival rate for localized Ewing's sarcoma is around 70-80%. For metastatic disease, the prognosis is not as good, but with modern treatments, the 5-year survival rate is around 30%. 

It is important to discuss your child's specific prognosis with your oncology team, as they can provide a more accurate picture based on the unique characteristics of the tumor. 

Screening and Prevention of Ewing's Sarcoma 

There are no routine screening tests for Ewing's sarcoma in the general population. The best way to ensure early detection is for parents and pediatricians to be aware of the symptoms. 

Prevention Strategies: 

• Since the causes are not fully known, there are no specific prevention strategies. 

• If your child has a persistent bone pain or a lump that doesn't go away, it is important to see a doctor promptly. 

For International Patients: Your Seamless Journey to Apollo Hospitals 

Apollo Hospitals is a leading medical destination for international patients seeking high-quality and affordable cancer care. Our dedicated International Patient Services team is here to ensure your entire experience is as smooth and comfortable as possible. We have extensive experience treating children and young adults with complex conditions, including Ewing's sarcoma. 

Our Services for International Patients Include: 

• Travel and Visa Assistance: We will provide you with a visa invitation letter and help with travel arrangements. 

• Airport Transfers: We will arrange for a car to pick you up from the airport. 

• Personalized Care: A dedicated patient coordinator will be your single point of contact, assisting with hospital admission, language interpretation, and any other needs you may have. 

• Accommodation: We can assist you with booking suitable accommodation for you and your family near the hospital. 

• Child Life Specialists: Our team includes child life specialists who can help your child and your family cope with the emotional and psychological challenges of a cancer diagnosis. 

• Post-Treatment Follow-up: We will stay in touch with you after your return home to ensure a smooth recovery.