Sarcomas are cancers of the connective tissue, (bone, muscle, fat, cartilage and soft tissue). There are many different types of sarcoma, and their cause is unknown. 99% of all sarcomas occur in children and young adults, and sarcomas represent 20% of all pediatric cancers. Each sarcoma individually, is so rare that they are considered an “orphan disease”.
Ewing’s sarcoma was first identified in 1921 by Dr. James Ewing. It is most often diagnosed between the ages of 10 and 20 years old, and accounts for 2 – 3 percent of all childhood cancers. In the US about 250 children and young adults are diagnosed with Ewing’s each year. It affects boys more than girls, and caucasians more than other races.
While the cause is unknown, researchers have discovered chromosomal changes in a cell’s DNA that can lead to the formation of Ewing’s. These changes are not inherited, and develop for no apparent reason. The change involves the fusing of genetic material between chromosomes # 11 and # 22. When a certain piece of chromosome # 11 is placed next to the EWS gene on chromosome # 22, the Ewing’s gene gets “switched on”. This activation leads to an overgrowth of the cells and the development of cancer. Current research is underway to better understand the basic biology of Ewing’s, and to eventually interfere with the “switching on” of the gene, or the pathways needed for cancer growth. However, across all age groups and 50 subtypes of cancer, sarcoma research received less than 1% of all funding from the National Cancer Institute. The funding for pediatric clinical trials, which are essential for developing new therapies, has declined each year since 2003.
Sources: Children’s Hospital Boston, Children’s Hospital of Philidelphia, Sarcoma Foundation of America, Dane-Farber Cancer Institute, Ewing’s Sarcoma Alliance
National Cancer Institute:
Liddy Shriver Sarcoma Initiative
Washington Musculoskeletal Tumor Center