What is Ewing's sarcoma?

This information has been written for patients, their families and friends and the general public to help you understand more about Ewing's sarcoma: what it is and the different types.

Ewing's sarcoma is a 'primary bone cancer'

Ewing's sarcoma is a rare cancer that can form in bone and soft tissue. It belongs to a group of tumours known as the Ewing's Sarcoma Family of Tumours.

The Ewing's family of tumours includes:

  • Ewing's sarcoma,

  • Extraosseous Ewing's sarcoma (starts in soft tissue rather than bone),

  • Askin tumour (Ewing's sarcoma of the chest wall),

  • Primitive Neuroectodermal Tumour (PNET).

The majority of Ewing's sarcomas (around 85%) start in the bones.

Ewing's sarcoma is the second most frequent primary malignant bone cancer found in young people after osteosarcoma and the third most frequent bone cancer overall. The cancer can occur anywhere in body but is mostly found in the pelvis, ribs and long bones, see figure 1.

Figure 1. Main Tumour Sites for Ewing's Sarcoma.

Compared to osteosarcoma, Ewing's sarcoma can arise more frequently from the diaphyseal (dy-AFEE-seal) area, also called the shaft of bones, see figure 2. Although, Ewing' sarcomas arise from elsewhere in the bone with almost equal frequency. Ewing's sarcoma is more commonly found in flat bones compared to osteosarcoma.

Figure 2, Basic Anatomy of Long Bones.

Because Ewing's sarcoma can spread quickly and has a tendency to comeback (recur), patients require treatment to the whole body (systemic) as well as the site of the primary tumour. Treatment is usually a combination of chemotherapy, surgery and radiotherapy. The treatment of Ewing's sarcomas whether they are found in soft tissues or bone is the same.

At diagnosis, 25-30% of patients have evidence that their cancer has spread to the lungs, other bones or to the bone marrow.

How does Ewing's sarcoma spread to other parts of the body?

Tumour cells can break away from the primary tumour and enter the blood supply or lymphatic (lim-FAT-ick) system. These tumour cells can travel to other parts of the body where they can form new tumours called secondary tumours or metastases (met-AS-tasis). The tumour can also spread through the bone.

The most common sites for secondary tumours are the

  • Lungs,

  • Other bones.

  • Bone marrow

Spread to lymph (lim-FF) nodes, liver and brain are rare.

Last reviewed: October 2010; Version: 1.1
Review due: October 2011

The authors and reviewers of this information are committed to producing reliable, accurate and up to date content reflecting the best available research evidence, and best clinical practice. We aim to provide unbiased information free from any commercial conflicts of interest. This article is for information only and should not be used for the diagnosis or treatment of medical conditions. BCRT can answer questions about primary bone cancers, including treatments and research but we are unable to offer specific advice about individual patients. If you are worried about any symptoms please consult your doctor.

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