SALT LAKE CITY — Researchers in Utah may have found a possible new treatment for an often deadly form of childhood cancer.
Ewings sarcoma, which is diagnosed in an average of 225 young men and young women each year in the United States, is a cancer that occurs primarily in the bone or soft tissue. It is often evidenced by localized pain in long bones, such as the femur (thigh), tibia (shin) or humerus (upper arm), and is believed to be caused by the actions of a cancer-causing protein (EWS/FLI) existing within the human genetic code.
Discovery of a new drug and the previously unknown mechanism behind it has led researchers at the Huntsman Cancer Institute to believe turning off specific genes could combat the pediatric cancer, which is the second-most common malignant bone tumor in children and adolescents, with diagnosis between the ages of 10 and 20.
"The beauty, if there's anything beautiful about a nasty disease like this, is that if we can inhibit EWS/FlI, we can inhibit this cancer, because EWS/FLI is the master regulator of Ewings sarcoma," said Dr. Stephen Lessnick, director of the institute's Center for Children's Cancer Research and a professor in the University of Utah School of Medicine's department of pediatrics.
Lessnick and his colleagues found that an enzyme, called lysine specific demethylase (LSD-1), interacts with EWS/FLI to turn off gene expression in Ewings sarcoma. By turning off specific genes, the complex causes Ewings sarcoma development.
"This makes LSD-1 an important target for the development of new drugs to treat Ewings sarcoma," he said. "For a long time, we've know that EWS/FLI works by binding to DNA and turning on genes that activate cancer formation, it was a surprise to find out that it turns genes off as well."