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Huntsman Cancer Institute

75 South 2000 East, Rm 211
Salt Lake City, UT 84112
United States

Background


Every cell generates and consumes energy to perform its functions. This is termed metabolism.

One intermediate step in sugar metabolism is a molecule called lactate. Traditionally, lactate was considered only a by-product of metabolism in tissues with low oxygen levels. This view was overturned when scientists learned that some cells with normal oxygen levels intentionally produce lactate and others consume it as a preferred fuel.

Our initial Alex's Lemonade Stand Foundation (ALSF) Epidemiology Award explored the prenatal, postnatal, and environmental risk factors that contribute to childhood cancer. We used the unique and powerful resource of the Utah Population Database (UPDB) to identify risk factors for pediatric cancer such as birth weight, birthplace, preeclampsia, Apgar score, and specific viral exposures.

Using a unique and powerful resource that combines genealogy from the 1800s with statewide cancer reporting from 1966 (the Utah Population Database or UPDB), we have identified very large families that have more cases of Ewing's Sarcoma than would be expected given the low rates of this cancer; we call these high-risk Ewing's Sarcoma pedigrees. The Ewing's Sarcoma cases in these families may all share a genetic change that they all inherited from a common ancestor, who is responsible for their cancer.

Symptoms related to cancer and its treatment are a major source of distress for children and adolescents with cancer. Children and adolescents who are hospitalized often report more symptoms than those in the outpatient setting. The hospital environment with its frequent disruptions and associated noise also can contribute to symptoms. Nurses are in an important position to influence the hospital environment in a manner that can worsen or improve symptoms.

Schiffman

Trede"Without ALSF's support we would not even be close to this discovery. Now because of your funds we are publishing the findings in Blood. The technician I was able to hire is absolutely fabulous and continues to make very nice progress on target identification." - Nikolaus Trede, MD/PhD, Huntsman Cancer Institute

Ewing sarcoma is a highly malignant bone cancer that occurs mostly in children and teenagers. Conventional treatment for Ewing sarcoma consists of multi-drug chemotherapy and surgery and/or radiation. These intensive treatments are toxic and have severe side effects and yet failed to improve the overall cure rate. New and effective therapies are needed.

Ewing's sarcoma is an aggressive bone tumor of children and young adults. Current treatment for this disease involves intensive chemotherapy, along with surgery and/or radiation therapy. These treatments are associated with serious short- and long-term side effects. Even with such intensive treatment, the overall 5-year cure rates for Ewing's sarcoma are on the order of 50%. There are two important unmet clinical needs for Ewing's sarcoma: improved diagnostic approaches to molecularly-characterize tumors, and improved therapies that target tumor-specific abnormalities.

Background


Protocols for the treatment of childhood cancers are beginning to incorporate our current knowledge of cancer genetics. For example, certain DNA mutations in a child's cancer might indicate that the disease will be difficult to cure. In such cases, curative treatments will need to be more intense. Conversely, children with mutations which indicate their disease is easier to cure can be spared high doses of chemotherapy. By this approach doctors hope to maximize cure rates while minimizing toxic side-effects, which can be severe.