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Ewing sarcoma
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Ewing sarcoma

Ewing sarcoma is a cancerous tumor of the bones or soft tissues that primarily affects children and young adults, driven by specific genetic mutations.

Overview

Ewing sarcoma is a cancerous tumor that occurs in bones or soft tissues, such as cartilage or nerves. There are several types of Ewing sarcoma, which are considered related because they share similar genetic causes. These types can be distinguished by the tissue in which the tumor develops:

  • Ewing sarcoma of bone: A bone tumor that accounts for approximately 87 percent of cases. It usually occurs in the thigh bones (femurs), pelvis, ribs, or shoulder blades.
  • Extraosseous (or extraskeletal) Ewing sarcoma: Tumors that develop in the soft tissues around bones, such as cartilage.
  • Peripheral primitive neuroectodermal tumor (pPNET): Tumors that occur in nerve tissue and can be found in many parts of the body.
  • Askin tumor: A specific type of pPNET that is found in the chest.

It is common for Ewing sarcoma to spread (metastasize) to other parts of the body, most frequently to the lungs, other bones, or the bone marrow.

Symptoms

Ewing sarcoma most often occurs in children and young adults. Affected individuals typically experience the following symptoms:

  • Stiffness, pain, swelling, or tenderness of the bone or surrounding tissue
  • A lump near the surface of the skin that feels warm and soft to the touch
  • A persistent fever that does not go away (often seen in children)
  • Weakening of the involved bone, which can lead to a broken bone with no obvious cause

Frequency

Approximately 3 per 1 million children are diagnosed with Ewing sarcoma each year. In the United States, an estimated 2

Frequently Asked Questions

combination of MRI (to define the local tumor size, bone involvement, and soft‑tissue extension) and a whole‑body PET/CT scan (to detect distant metastases, especially in the lungs and bone marrow) provides the most comprehensive staging information.
core needle or open surgical biopsy is required to obtain tissue. Pathologists look for small round blue cells and confirm the diagnosis with molecular testing for the characteristic EWS‑FLI1 translocation. Without this genetic confirmation, the tumor could be mistaken for other small‑cell cancers.
Metastatic disease adds systemic chemotherapy intensification and may include high‑dose chemotherapy with stem‑cell rescue. Radiation to lung metastases is considered if they are few. Survival rates drop compared with localized disease, but aggressive multimodal therapy can still achieve long‑term remission in many patients.
Treatment typically involves multi‑agent chemotherapy (often vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide) given in cycles over 6–9 months, followed by local control with surgery, radiation, or both. The entire protocol usually spans about 9–12 months.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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