Ewing sarcoma

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Ewing sarcoma is a type of cancer that can start in bone and sometimes soft tissue. It is most commonly found in children, teens and young adults. Learn about the signs and symptoms and how it is diagnosed and treated.

Key points

Ewing sarcoma is a rare type of cancer affecting bones or soft tissue.
It is most commonly found in children, teens and young adults between ages 10 and 20.
The most common symptom of Ewing sarcoma is pain (especially when it starts in the bone).
This pain may initially be thought to be related to sports injury, growing pains or an inflammatory condition.

Introduction

What is Ewing sarcoma?

Ewing sarcoma is a rare type of cancer which can affect any bone and sometimes soft tissue. The bones most commonly affected are the leg and arm bones, pelvis, ribs, spine and sternum. Ewing sarcoma is seen most often in pre-teens, adolescents and young adults between the ages of 10 and 20. In patients where the cancer has not spread the five-year survival rate is greater than 70%.

Signs and symptoms

Symptoms of Ewing sarcoma

Symptoms of Ewing sarcoma include:

swelling or a lump at the site of the tumour
pain near the site of the tumour
pain from an injury that is greater than expected and does not get better with time

Often patients are very well except for a painful bump.

Causes, risk factors and prevalence

What causes Ewing sarcoma?

There is currently no known cause for Ewing sarcoma. It is not hereditary. It is the second most common primary bone tumour.

Who gets Ewing sarcoma?

Ewing sarcoma is most commonly seen in pre-teens, adolescents and young adults between the ages of 10 and 20.

Spread, phase, course of disease

Spread of Ewing sarcoma

Ewing sarcoma can spread or metastasize to other parts of the body. At diagnosis, one in four people will have a tumour that has spread to another part of their body. The most common site of spread is to the lungs.

Diagnosis

Diagnosing Ewing sarcoma

Initially, the symptoms of Ewing sarcoma can be diagnosed as a sports injury, growing pains or an inflammatory condition. However, if symptoms cannot be fully explained, persist or change rapidly, see your doctor immediately for further assessment.

Your child’s doctor will do a complete history and physical examination that includes:

if there is a lump or swelling, and whether it has changed in size
the amount of pain, whether it is constant or increasing in intensity
if there is an unexplained limp or problem using their limbs

Your child’s doctor will order X-rays, a CT scan or an MRI of the area of concern.

A biopsy of the mass will be done to confirm the diagnosis.

To check to see if the cancer has spread, a CT scan of the lungs and a scan of the bones will also be done.

Treatment

Treatment for Ewing sarcoma

The standard treatment for Ewing sarcoma includes both chemotherapy as well as some form of local control. Local control includes either surgery or radiation or both. The chemotherapy generally lasts for 28 to 30 weeks in total. Both chemotherapy and local control are needed in order to treat Ewing sarcoma. There are both short-term and long-term side effects of the treatment.

Resources

For additional information about Ewing sarcoma please visit the following resources.

Find more information about Ewing sarcoma at the National Cancer Institute.
Read an article about how children’s bone cancers could remain hidden for years before diagnosis.
A guide for physicians providing information about Ewing sarcoma.

This page was supported and created in collaboration with the Ewings Cancer Foundation of Canada.

References

The Canadian Childhood Cancer Surveillance and Control Program. (August 2004). Diagnosis and Initial Treatment of Cancer in Canadian Adolescents 15 to 19 Years of Age, 1995–2000. Retrieved from http://publications.gc.ca/collections/Collection/H39-4-38-2004E.pdf
Public Health Agency of Canada. (October 2017). Cancer in Young People in Canada: A Report from the Enhanced Childhood Cancer Surveillance System. Retrieved from https://www.canada.ca/content/dam/hc-sc/documents/services/publications/science-research-data/cancer-young-people-canada-surveillance-2017-eng.pdf
George, G., and Grimer, R. (2012). Early symptoms of bone and soft tissue sarcomas: could they be diagnosed earlier? Annals of The Royal College of Surgeons of England, 94, 261-266. doi: 10.1308/003588412X13171221590016
Grimer, R.J., and Briggs, T.W. (2010). Earlier diagnosis of bone and soft-tissue tumours. The Journal of bone and joint surgery. British volume, 92, 1489-92. doi: 10.1302/0301-620X.92B11.24326
Borinstein, S.C., Beeler, N., and Block, J.J., et al. (2013). A Decade in Banking Ewing Sarcoma: A Report from the Children's Oncology Group. Frontiers in oncology, 3, 57. doi: 10.3389/fonc.2013.00057
Bacci, G., Ferrari, S., and Longhi, A., et al. (2003). Therapy and survival after recurrence of Ewing's tumors: the Rizzoli experience in 195 patients treated with adjuvant and neoadjuvant chemotherapy from 1979 to 1997. Annals of oncology, 14, 1654-9. doi: 10.1093/annonc/mdg457
Leavey, P.J., Mascarenhas, L., Marina, N., et al. (2008). Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children's Oncology Group. Pediatric blood & cancer, 51, 334-8. doi: 10.1002/pbc.21618

Last updated: November 8th 2018