The Ewing family of tumors (EFT) comprise a spectrum of tumors that include Ewings sarcoma of bone, extraosseous (outside of the bone) Ewings sarcoma and primitive neuroectodermal tumors. This family of tumors occur usually in children and adolescents between the ages of ten to twenty and comprise approximately 4% of cancers in children and adolescents. Ewings sarcoma of bone is the second most common malignant bone tumor in this age category.
EFT have been found to have a common chromosomal abnormality on chromosome 22 with involvement of other chromosomes, especially chromosome 11 and less frequently chromosome 21, 7 and 17. Other chromosomal aberrations have also been found. These chromosomal alterations lead to changes in the effected cell which leads to abnormal cell growth and the development of EFT. These chromosomal alterations cause unique molecular alterations that can be used to aid in the diagnosis of EFT.
The standard approach to treating EFT is with surgery, chemotherapy and radiation. By using these modalities the cure rate for this group of tumors is approximately 65%. The question remains why certain children do better than others. The Nicholas Currey Fund is supporting research that is addressing this question. As mentioned above changes in chromosomes are thought to be responsible for the development of EFT. There is a gene, p53, that serves to suppress tumor development. Mutations in this gene often lead to tumor development. Preliminary data suggests that these mutations, and those in another closely related gene in the p53 pathway, may play adverse roles in EFT patients who do not respond well to chemotherapy. Through funds raised by the NCF, banked tumor tissue samples from patients with EFT who were treated by institutions of the Children’s Oncology Group will be tested to determine if patients with certain types of mutations in these genes fare worse than those without such mutations. If this is found to be true then these children and adolescents could eventually be offered newer and different treatments that might enhance their chances for cure.
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