What is ewing sarcoma

Last updated: April 1, 2026

Quick Answer: Ewing sarcoma is a malignant bone cancer that typically develops in the long bones of the legs and pelvis in children and young adults, usually between ages 10 and 20. It is the second most common primary bone cancer in children and can spread to other parts of the body if untreated.

Key Facts

Overview

Ewing sarcoma is a malignant tumor that develops in bone, most commonly in children and young adults aged 10-20 years old. It is the second most common primary bone cancer after osteosarcoma and represents approximately 9% of all primary bone cancers. The disease is characterized by rapid growth and a tendency to spread to other tissues, particularly the lungs.

Causes and Risk Factors

Ewing sarcoma is caused by a specific chromosomal abnormality where genetic material from chromosome 22 fuses with material from chromosome 11, creating an abnormal EWSR1-FLI1 fusion gene. This fusion produces an abnormal protein that causes bone cells to become cancerous. The exact reason why this translocation occurs is not fully understood, and there are no established preventable risk factors. The disease appears to arise randomly and is not hereditary in most cases.

Symptoms and Diagnosis

Common symptoms include localized bone pain and swelling in the affected area, sometimes accompanied by fever. Patients may develop a palpable mass or experience limited mobility. Diagnosis typically involves X-ray imaging followed by MRI for detailed evaluation, and confirmation through biopsy. CT scans are often used to detect metastatic disease in the lungs and other organs.

Treatment Approach

Modern treatment uses a multimodal approach combining neoadjuvant chemotherapy (before surgery), surgical resection of the tumor, and sometimes radiation therapy. Standard chemotherapy regimens include doxorubicin, cyclophosphamide, etoposide, ifosfamide, and cisplatin. Surgery aims to completely remove the tumor while preserving limb function when possible. Radiation is typically reserved for cases where surgical margins cannot be achieved or for metastatic disease.

Prognosis and Survival

Patients with localized Ewing sarcoma treated with multimodal therapy achieve 5-year overall survival rates of 65-75%. Outcomes are significantly worse for patients presenting with metastatic disease, with 5-year survival rates around 20-30%. Prognostic factors include tumor size, location, and response to chemotherapy.

Related Questions

How is Ewing sarcoma different from osteosarcoma?

While both are malignant bone cancers occurring in children, osteosarcoma produces bone matrix and typically occurs in osteoblasts, whereas Ewing sarcoma arises from bone marrow cells. They have different chromosomal abnormalities and treatment protocols.

Can Ewing sarcoma be cured?

With modern multimodal treatment, many patients with localized Ewing sarcoma achieve long-term remission and cure. However, metastatic disease remains challenging, with lower cure rates but still achievable with aggressive treatment.

What are the side effects of Ewing sarcoma treatment?

Chemotherapy can cause nausea, hair loss, infection risk, and heart damage. Surgery may affect limb function or require amputation in some cases. Radiation carries risks of secondary cancers and tissue damage.

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Sources

  1. National Cancer Institute - Ewing Sarcoma Treatment CC-BY-4.0
  2. Wikipedia - Ewing's Sarcoma CC-BY-SA-4.0