Utah researchers discover possible new treatment for a childhood cancer (+video)

Published: Tuesday, Nov. 27 2012 7:25 p.m. MST

Chante Wouden survived two bouts of Ewing's sarcoma as a child, using experimental treatments. Researchers at the Huntsman Cancer Institute and the University of Utah have discovered a possible new treatment.

Scott G Winterton, Deseret News

SALT LAKE CITY — Chante Wouden was nearly given a death sentence with the diagnosis of Ewing's sarcoma at age 3.

A treatment cocktail of various chemotherapy drugs didn't completely kill the cancer in her hip the first time around, and at age 5, she found herself again living at Primary Children's Medical Center — this time with cancer in her head and back.

"The world outside of that hospital ceases to exist because you are so focused on battling for your life," the now-31-year-old survivor said Tuesday. "Your family becomes other patients who are also fighting for their lives and the doctors and nurses who are fighting with you."

A new discovery by researchers at the Huntsman Cancer Institute and the University of Utah may someday spare many more lives of Ewing's sarcoma patients. The findings and details of a possible new treatment for the often deadly childhood cancer, appears in Tuesday's online issue of the journal Oncogene.

Wouden had few options, but an experimental treatment was available. With a mother who strongly believed in the power of positive thinking, "we focused on life and living," she said.

"Looking back, I know the treatments I received kept me alive long enough to receive new treatments," Wouden said. "The treatment I received at age 5 wasn't available when I was 3. It's like jumping from one stepping stone in a river to another."

The ongoing research, she said, always gave and continues to give her hope. She still deals with the latent effects of the cancer and the cancer treatment she received as a child, but she remains optimistic that medical research will continue to keep her alive.

Ewing's 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 or swelling 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.

However, the cause is not fully understood and risk factors or prevention measures are not known.

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 Ewing 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 Ewing's sarcoma. By turning off specific genes, the complex causes Ewing's sarcoma development.

"This makes LSD-1 an important target for the development of new drugs to treat Ewing 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."

Dr. Sunil Sharma, director of Huntsman's Center for Investigational Therapeutics, had already focused on LSD-1 as a possible target for new cancer treatments and had been working for several years to design drugs that would inhibit its actions.

He said the enzyme was important "for regulation of a variety of properties in several different cancers, including acute leukemias, breast and prostate cancers."

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