Based on their findings, the study authors recommend:
Careful and ideally centralized pathologic review for all individuals with OSCST tumors DICER1 testing for all those with SLCT and GAB and Consideration of DICER1 testing for patients with other OSCSTs.
“Genetic testing may be useful for screening and diagnosing entire families if one family member tests positive for a DICER1 mutation, especially to determine if they are at risk for PPB. When we know who is at risk, we can protect all children in a family,” Dr. Hill says.
“Ultimately we may be able to cure this deadly lung cancer, PPB, by identifying and performing computed tomography scans on people who are at risk, so we can catch these cancers early.”
Dr. Hill thinks future research may study children whose cancer was not detected early or has become resistant to chemotherapy. They also may explore ways to restore normal controls in cancer cells, so they follow normal paths of development, for the purpose of developing targeted treatments with fewer side effects than
current therapies.
In addition to Dr. Hill, other Children’s National study co-
authors include Amanda Field, M.P.H., Department of Pathology; Weiying Yu, Ph.D., Department of Pathology; and Joyce Turner, director of the Cancer Genetic Counseling Program in Children’s Rare Disease Institute.
Other members of the study team are experts from the International Ovarian and Testicular Stromal Tumor Registry, Children’s Minnesota, Washington University Medical Center, Carolinas Health Care System, University of Texas MD Anderson Cancer Center, Harvard Medical School, University of Colorado School of Medicine, Clinic of Pediatrics (Dortmund, Germany), National Cancer Institute and Dana-Farber Cancer Institute.
Research reported in this story was supported by the National Institutes of Health under award number NCI R01CA143167, The Parson’s Foundation, St. Baldrick’s Foundation, Pine Tree Apple Tennis Classic Foundation, Hyundai Hope on Wheels, the Randy Shaver Cancer Research and Community Fund, the German Childhood Cancer Foundation and the Intramural Research Program of the Divisions of Cancer Epidemiology and Genetics, National Cancer Institute.
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https://innovationdistrict.childrensnational.org/new-clues-to-detect-rare-pediatric-cancers/
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