Wilms tumor is the most common type of kidney cancer found in children and most often occurs under age 10. It’s fairly rare—only about 500-600 children are diagnosed every year—and treatable with a high success rate. But many childhood survivors of Wilms tumors deal with lifelong consequences from the toxicity of the treatment. They are more likely to develop future cancers and less likely to go to college, hold a job, or marry, than kids who have not had childhood cancer.
A pediatric oncologist at the University of Texas Southwestern Medical Center wants to improve treatments for Wilms tumors to give survivors the same potential for a full life as their peers. Dr. Kenneth Chen, M.D., has been at UT Southwestern since he started medical school, and stayed to become an instructor in pediatrics. A First-Time Tenure-Track Award from CPRIT in 2019 has allowed this talented physician-scientist to remain in Texas as an assistant professor of pediatrics.
Dr. Chen is interested in a type of genetic material, called microRNAs, which help regulate the expression of other genes during development. In about a fifth of Wilms tumors, there are mutations in the genes coding for the microRNAs. Dr. Chen wants to understand how these mutations are involved in cancer development and look for ways to therapeutically intervene.
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Wilms tumor is the most common type of kidney cancer found in children and most often occurs under age 10. It’s fairly rare—only about 500-600 children are diagnosed every year—and treatable with a high success rate. But many childhood survivors of Wilms tumors deal with lifelong consequences from the toxicity of the treatment. They are more likely to develop future cancers and less likely to go to college, hold a job, or marry, than kids who have not had childhood cancer.
A pediatric oncologist at the University of Texas Southwestern Medical Center wants to improve treatments for Wilms tumors to give survivors the same potential for a full life as their peers. Dr. Kenneth Chen, M.D., has been at UT Southwestern since he started medical school, and stayed to become an instructor in pediatrics. A First-Time Tenure-Track Award from CPRIT in 2019 has allowed this talented physician-scientist to remain in Texas as an assistant professor of pediatrics.
Dr. Chen is interested in a type of genetic material, called microRNAs, which help regulate the expression of other genes during development. In about a fifth of Wilms tumors, there are mutations in the genes coding for the microRNAs. Dr. Chen wants to understand how these mutations are involved in cancer development and look for ways to therapeutically intervene.
Childhood cancers arise from embryonic tissues that have gone awry in development. While the mutations that cause Wilms tumor are kidney-specific, there are similar mutations in a wide variety of other childhood cancers.
Dr. Chen is also interested in a family of tumors that arise from an inherited genetic syndrome called DICER1. Children with a missing or altered copy of a particular gene are prone to developing cancers throughout their lifetimes, especially in childhood. The cancers can arise in a variety of different organs but are thought to share a common mechanism through microRNA mediation of developmental genes.
“In DICER1 syndrome, patients are born with one copy of a mutated gene, and the tumors themselves develop another copy,” Dr. Chen says. “And as we are learning more about these mutations, we find they have noncancerous manifestations as well, like bigger heads, higher blood pressure, and a risk of thyroid disease.”
Current treatments target DICER1 syndrome cancers based on the organ they develop in—muscle tumors are treated like other muscle tumors, or ovarian tumors are treated as an ovarian tumor—even though they are biologically different because of the DICER1 mutation.
“The dream is to have a strategy for treating DICER1 syndrome cancers regardless of the tissue in which they develop,” Dr. Chen says, “based on their molecular manifestation rather than the organ where they develop.”
As with Wilms tumors, the treatment would be directed at the microRNA dysfunction rather than the underlying genetic mutation.
Dr. Chen is helping to write the first clinical trial for the most common lung cancer in children with DICER1 syndrome, pleuropulmonary blastoma. Because DICER1 syndrome is so rare, he and his colleagues are developing an international collaboration in order to enroll enough patients.
“With these really rare tumors one of the ways to make an impact is for everyone to work together,” he says, adding, “I hope the next trial will come out of my research.”
Dr. Chen received his undergraduate degree in biomedical engineering from The Johns Hopkins University and his M.D. from UT Southwestern, where he was a resident in pediatrics and a pediatric oncology fellow. He became an instructor in pediatric oncology at UT Southwestern in 2016.
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