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


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.”

The two researchers teamed up to test Ewing’s sarcoma tissue cultures, which proved beneficial. They are now working together in animal testing, aiming at future human trials.

Wouden said she cheers for any advancement in cancer research, however seldom they may come along.

“When you see how fragile and temporary life is, you also see more clearly how you want to cherish it and how you want to spend it,” she said.

Wouden lives in Bountiful and works at her “dream job,” producing various media products for The Church of Jesus Christ of Latter-day Saints, and is planning to marry her fiance in February.

“There are so many forms of cancer, it could be so overwhelming that we all just give up,” she said. “I am a firm believer in the role that medicine plays in healing and that it played in healing me. It was integral to my survival. I owe my life to it.”

The Ewing’s sarcoma-related research is funded by a variety of sources, including grants from the National Institutes of Health and the Howard Hughes Medical Institute.

“This is a great example of how collaboration between the therapeutics and basic science programs can lead to new treatments for patients — one of Huntsman Cancer Institute’s highest goals,” Sharma said.

Existing treatment options for Ewing’s sarcoma include surgery to remove the tumor, chemotherapy, radiation and ultimate amputation of the affected arm or leg, among others. Prognosis greatly depends on the extent of the disease, size and location of the tumor, response to therapy, age and overall health, tolerance of medications and procedures, but it also relies heavily on new developments in treatment.

E-mail: wleonard@desnews.com

Twitter: wendyleonards

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