Ewing Sarcoma - Sri Shankara Cancer Hospital & Research Centre

Ewing Sarcoma: A Comprehensive Overview

General Information

Ewing sarcoma, also known as an Ewing tumour, is a rare type of cancer that typically develops in the bones or the soft tissues surrounding the bones. It most commonly starts in the pelvis, chest wall, or the long bones of the legs, such as the thighbone (femur) or shinbone (tibia). In fewer instances, these tumours can grow in the skull or the flat bones of the trunk.

This cancer is considered rare, accounting for approximately 1% of all childhood cancers. While it primarily affects teenagers, it can also be found in younger children, young adults, and even older adults, with about 40% of cases diagnosed in the adult population.

Ewing sarcomas are caused by genetic changes that occur after birth; they are not hereditary and do not run in families.

Types of Ewing Sarcoma

Although they may look slightly different, both main types share the same underlying genetic changes and are treated using similar approaches:

Ewing Sarcoma of the Bone

The most common form, originating within the bones themselves.

Extraosseous Ewing Tumour (EOE)

A tumour that develops in the soft tissues adjacent to the bones.

Symptoms

The symptoms of Ewing sarcoma can vary greatly depending on the tumour's location and size. Often, the initial symptom is pain at the tumour site, which may become more severe at night or during physical activity. The pain may be a result of the tumour growth or a bone fracture due to the tumour weakening the bone.

Early Symptoms

Early signs can often be subtle and may be confused with minor sports injuries or common "growing pains." These can include:

  • Mild or intermittent pain in a bone or joint.
  • Swelling or tenderness in the affected area.
  • General fatigue or a feeling of being unwell.
  • Limping, if the cancer is in the leg.

Late Symptoms

As the tumour grows, symptoms tend to become more pronounced:

  • A noticeable lump or mass where the tumour is located.
  • Persistent or worsening pain, sometimes severe.
  • Bones breaking following minor impact or normal activity.
  • Unexplained symptoms such as fever, night sweats, or unintentional weight loss.
  • Weakness, numbness, or paralysis in the arms or legs if the tumour is pressing on the spinal cord.
  • Shortness of breath, which may occur if the cancer has spread to the lungs.

It is important to note that many of these symptoms overlap with other health conditions, such as infections or injuries. Therefore, seeing a doctor for a professional diagnosis is crucial. You should contact a healthcare professional promptly if you experience persistent bone or joint pain, a new lump that does not go away, or unexplained fever or weight loss.

Diagnosis

A definitive diagnosis of Ewing sarcoma relies on a biopsy of the tumour. It is strongly advised that this initial biopsy be performed at the cancer centre where the patient intends to receive treatment. This ensures accurate sampling by an experienced specialist and helps minimise the risk of complications.

At SSCHRC, our specialised sarcoma teams—comprising surgeons, oncologists, radiologists, pathologists, and genetic counsellors—have extensive expertise in diagnosing this rare cancer. This coordinated, multidisciplinary approach ensures the most precise diagnosis and the development of the most effective treatment plan.

Diagnostic Tests

If symptoms suggest Ewing sarcoma, a doctor will conduct a thorough physical examination and review the patient's medical and family history. A combination of tests is used to confirm the diagnosis, determine the tumour's exact location and size, and check for cancer spread:

Biopsy

This is the only way to definitively diagnose Ewing sarcoma. A small sample of bone or soft tissue is removed and examined for cancer cells.

  • Needle Biopsy: A hollow needle is inserted into the tumour, often guided by scans, to remove a tissue sample.
  • Open/Surgical Biopsy: An incision is made to remove a small or, rarely, the entire tumour.
  • Note: Whenever possible, the surgeon who performs the biopsy should also perform the subsequent tumour removal surgery for precise planning and reduced complication risk.

Imaging Tests

These scans help determine the tumour's exact location, size, and whether it has spread:

  • CT or CAT Scans: Provide detailed X-ray images to assess the tumour and nearby structures.
  • MRI Scans: Offer high-resolution images of soft tissue involvement and bone marrow.
  • PET Scans: Used to detect potential spread of cancer to other organs.
  • X-rays: Show abnormalities or fractures in the bone.

Genetic Testing

Testing is often performed on the biopsy tissue to confirm the diagnosis and differentiate Ewing sarcoma from other types of tumours.

Blood Tests

Bloodwork is used to evaluate the patient's overall health, organ function, and readiness for treatment.

Treatment

The experts at SSCHRC personalise treatment plans for every patient with Ewing sarcoma. A highly specialised team provides continuous care to maximise effectiveness while carefully considering the impact on growth and development, particularly for younger patients.

Treatment for Ewing sarcoma typically involves a combination of therapies:

Chemotherapy

  • Chemotherapy drugs are used to kill cancer cells, control their growth, or relieve symptoms.
  • It is usually the first step in treatment because Ewing sarcoma often spreads microscopically to other parts of the body early on.
  • It is generally followed by surgery and/or radiation therapy, with additional chemotherapy often given afterwards.
  • SSCHRC uses the latest chemotherapy protocols to balance treatment effectiveness with the management of side effects.

Surgery

  • The aim of surgery is to remove the tumour along with a margin of surrounding healthy tissue to ensure all cancer cells are removed.
  • Surgery for Ewing sarcoma often focuses on limb-sparing techniques, preserving limbs and joint function whenever possible.
  • Specialised orthopedic surgeons at SSCHRC are skilled in complex procedures that may involve replacing removed bone with a bone graft or an internal prosthesis.

Radiation Therapy

  • Radiation therapy uses powerful, focused beams of energy to destroy cancer cells.
  • It may be used when surgery is not feasible, when it would cause excessive loss of function, or in combination with surgery to eliminate any remaining cancer cells.
  • SSCHRC radiation oncologists utilise advanced techniques to accurately target the tumour while minimising damage to healthy tissue.

Targeted Therapy and Clinical Trials

  • Targeted therapy drugs are designed to interfere with specific molecules that cancer cells need to survive and spread.
  • For some patients, newer and cutting-edge treatments may be accessible through clinical research studies, providing access to therapies not yet widely available.
  • SSCHRC is one of the world's leading centres offering such advanced treatment options.
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