Definition

Ewing sarcoma is a malignant tumor (cancer) that originates in the bone or soft tissue around the bone. It was first identified by James Ewing in 1921. Ewing sarcoma is the second most common bone cancer in children and young adults, with its peak occurrence during adolescence.

This cancer commonly affects the pelvic bone, femur, and axial skeleton, such as the skull, spine, and ribs. However, Ewing sarcoma can occur in almost any bone or soft tissue. Due to its malignant nature, it can spread to other organs in the body.

Causes

The exact cause of Ewing sarcoma is not fully understood. It is unclear which cells give rise to cancer. What is known is that a fragment of genetic material from chromosomes 11 and 12 is exchanged and attaches to different chromosomes. This genetic abnormality is not inherited from the parents but occurs after the child is born.

This genetic mutation disrupts the cell life cycle, causing normal cells, which would typically die off, to continue growing and invading healthy tissue. These abnormal cells can also spread to other organs in the body.

Risk Factor

There is no clear scientific association between Ewing sarcoma and specific risk factors such as:

• Environmental exposure
• Drug exposure
• Radiation history
• Family history of cancer

Factors that make someone more susceptible to Ewing sarcoma are still uncertain. However, from the age range of patients, it is more commonly found in teenagers and young adults, with a median age of 15. The highest number of new cases is seen in patients aged 10-15 years, with males affected more often than females, at a ratio of 3:1.

Symptoms

A tumor may exist for several months before becoming large enough to cause noticeable symptoms. Ewing sarcoma can affect any bone, but it most commonly occurs in the femur, pelvis, and chest bones. In some cases, the first symptom a patient experiences is the presence of a large mass or lump in the body.

Patients with Ewing sarcoma often present with symptoms such as pain, stiffness, or swelling in the affected bone. These symptoms may last for several weeks to months. More than 50% of patients experience intermittent pain, which worsens at night.

In addition to bone-related symptoms, other signs may include:

• Fever
• Significant weight loss without dieting or regular exercise
• Unexplained fatigue
• Respiratory issues related to the lungs or pleura, if the tumor has spread extensively

Diagnosis

Understanding the clinical course and conducting a comprehensive physical examination are crucial in diagnosing Ewing sarcoma. The doctor will ask the patient or their family about symptoms, medical history, and any treatments that have been administered.

The doctor will perform a thorough physical examination and check for lumps or masses, changes in body shape, and the degree of pain. If the cancer has spread to organs like the lungs, abnormal breath sounds may be detected.

Cancer usually requires additional tests to confirm the diagnosis. These may include:

• Imaging tests to get a clear view of the affected area, such as:
• X-rays
• CT scans
• MRI
• Bone scans
• Other tests to determine the stage of cancer and find out if cancer cells have spread to other organs, such as:
• Blood tests
• Bone marrow biopsy
• Chest CT scan
• MRI of the spine or pelvis
• Tissue biopsy, where the doctor takes a sample of the suspected cancerous tissue for microscopic examination

Management

Before starting treatment, patients may be offered fertility counseling. The treatment strategy is planned based on the examination results, cancer stage, and patient condition. Ewing sarcoma treatment typically involves a team of specialized doctors, with the main therapies being chemotherapy, surgery, and radiotherapy, often used in combination.

Chemotherapy

This drug therapy is used to kill tumor cells. It is typically used to shrink the tumor, making it easier to remove surgically. Patients receive the medication through a small catheter inserted into a vein in the chest.

Surgery

Surgery is performed to remove the primary tumor and any smaller tumors that have spread to other organs. The tumor is removed along with some surrounding healthy tissue to prevent recurrence. Surgeons may also reconstruct joints, bones, or soft tissue. The body’s ability to function after surgery depends on how much joint and muscle tissue can be preserved during tumor removal. In some cases, amputation of a limb may be necessary to ensure that the tumor and surrounding tissue are fully removed.

Radiotherapy

Radiation therapy is effective in destroying and shrinking tumor cells. It is typically used when surgery cannot fully remove the tumor or when surgery could result in the loss of function in the affected area.

Complications

Ewing sarcoma can lead to sudden bone fractures, even without injury to the affected area. If diagnosed late, the cancer may spread to other organs, making treatment and recovery more challenging. Ewing sarcoma commonly spreads to the lungs and other bones. Death can also be a complication of this cancer.

Treatment procedures can cause both short-term and long-term side effects. It is essential to discuss these with your doctor and be aware of any potential side effects so that you can seek medical attention if necessary.

Prevention

There is no specific way to prevent Ewing sarcoma. Patients and their families should be educated about the importance of following all treatment modalities to prevent complications.

As with other cancers that have idiopathic (unknown) and multifactorial causes, maintaining a healthy lifestyle is the best prevention.

When to See a Doctor?

The most common symptoms of Ewing sarcoma include pain, swelling, or stiffness in the affected bones for several weeks. Although this cancer is rare, it is most common in teenagers and young adults. If your symptoms are concerning, it's a good idea to consult a doctor. Early diagnosis can improve survival rates for those affected.

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