Ewing's Sarcoma: What you need to know about this rare peadiatric bone cancer

Certain medical conditions begin with abnormal cell growth in bones or soft tissues, affecting both children and adults. These conditions often arise without a clear cause, driven by random genetic mutations. They can manifest in various parts of the body and may lead to symptoms like pain, swelling, or the development of lumps. Early diagnosis and treatment are crucial for managing these conditions and improving outcomes.

Ewing sarcoma is a rare type of cancer that originates in the bones or surrounding soft tissues. Although it predominantly affects children and young adults, it can develop at any age. Ewing sarcoma most commonly develops in the bones of the legs and pelvis, but it can also appear in other bones or soft tissues such as the chest, abdomen, or arms.

The exact cause of Ewing sarcoma remains unclear, but it is linked to random genetic changes in cells, not inherited from parents. These mutations occur spontaneously and lead to the development of cancerous growths.

Symptoms
Ewing sarcoma symptoms typically manifest around the affected bone and may include:
• A lump or swelling in the arm, leg, chest, or pelvis
• Persistent bone pain
• Fractures occurring without significant trauma
• Pain, swelling, or tenderness near the tumor site
• Fever
• Unexplained weight loss/Fatigue

Diagnosis
To diagnose Ewing sarcoma, healthcare providers will assess your child's medical history and symptoms, conduct a physical examination, and may refer your child to a specialist, such as an orthopedic oncologist. Diagnostic tests may include:
• X-ray: Using radiation to capture detailed images of bones and tissues for accurate diagnosis
• Blood tests: Help assess overall health and detect signs of sarcoma
• CT scan: Produces detailed images of the body through a series of X-rays
• MRI: Uses magnets and radio waves to create detailed body images
• Bone scan: A radioactive tracer highlights areas of bone that may have cancer
• PET scan: Detects cancer cells by highlighting areas where radioactive sugar accumulates
• Bone marrow aspiration/biopsy: Tests whether cancer has spread to the bone marrow
• Tumor biopsy: A sample of the tumor is tested for cancer cells and genetic markers

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Treatment Options
Treatment for Ewing sarcoma depends on its stage and other factors and may include:
• Surgery: Removes the tumor and surrounding tissue, often preserving the affected limb. This is also referred to as limb-salvage or a limb-sparing surgery.
• Chemotherapy: Uses powerful drugs to kill cancer cells, often before or after surgery. Chemotherapy is administered in cycles with intervals of rest. It's common for multiple chemotherapy drugs to be used in combination.
• Radiation therapy: Employs high-energy X-rays to destroy cancer cells, particularly when surgery isn't sufficient.
• High-dose chemotherapy with stem cell transplant: Used in cases with a high risk of recurrence, this treatment involves replacing damaged bone marrow with healthy stem cells.

Key Points to Remember
• Although a rare scenario, Ewing sarcoma usually presents in the bones or soft tissues
• A biopsy is necessary to confirm a diagnosis
• Treatment typically involves chemotherapy, surgery, or radiation, and may include high-dose chemotherapy with stem cell transplants
• Long-term follow-up care is crucial to monitor for complications from the disease and its treatment

In conclusion, Ewing’s sarcoma is a rare but aggressive pediatric bone cancer that necessitates prompt diagnosis and a multifaceted treatment approach. Despite its uncertain origins, advancements in medical treatments have significantly improved the outlook for patients. However, the journey doesn't end with treatment; long-term follow-up is essential to monitor for recurrence and manage any complications that arise. With early intervention and comprehensive care, many children and young adults with Ewing’s sarcoma can achieve positive outcomes, paving the way for a brighter future.

(Dr. Kapil Raut, Consultant- Medical Oncology, HCG Cancer Centre, Nagpur)