My daughter Bethan was diagnosed with primary (localised) Ewing’s sarcoma in her left humerus in 2012. I like ...
Ewing sarcoma is the second most common type of bone tumour to affect children, after osteosarcoma.
Whilst almost all cases of osteosarcoma occur in either the long bones of the legs (the femur and tibia) or the long bone of the arms (the humerus), Ewing sarcoma occurs in a broader range of sites.
Ewing sarcoma is actually a group of four different types of cancer, known collectively as the Ewing family of tumours (EFT). Ewing sarcoma of bone is the most common of these. Extraosseous Ewing sarcoma occurs in the soft tissue surrounding bone; peripheral primitive neuroectodermal tumour (PNET) can be found in either the bone or the soft tissue and Askin tumour occurs in the chest wall.
The cause of EFTs is unknown but it is thought that they may be linked to periods of rapid bone growth, explaining why they are seen more often in teenagers.
In Great Britain, bone sarcoma accounts for 4% of all childhood cancers. Incidence rates are very similar in both sexes and increase steadily with age; overall, bone sarcoma accounts for 9% of all cancers diagnosed in 10-14 year-olds. The most common types of bone sarcoma are osteosarcoma (accounting for 53% of cases) and Ewing tumour and related bone sarcomas (42% of cases). Around 58 children per year are diagnosed with Ewing sarcoma of the bone in Great Britain each year.
Up to 40% of childhood cases occur in the femur and tibia. Substantial numbers of cases arise in the pelvis (23%), long bones of the arms (11%), ribs, sternum and clavicle (9%) and spinal column (8%).
Ewing sarcoma has a lower incidence in children than osteosarcoma. The majority of cases are in the 10-14 year age group but the predominance of older children is less pronounced than for osteosarcoma. Up to the age of six years, Ewing sarcoma is the most common type of malignant bone tumour. Incidence is similar in boys and girls up to age 10 when a male excess begins to emerge.
This male excess emerges later in osteosarcoma (around 15-16 years). In addition to the Ewing tumours of bone, around 16 children a year are diagnosed with the soft tissue types of Ewing sarcoma.
The overall five year survival rate from Ewing sarcoma family tumours of bone is 66%. For extraosseous tumours, the survival rate is lower at 58%.
An individual’s prognosis depends on the size of the tumour, site, whether it has spread and response to treatment.Back to top