Scientist Seeks to Understand the Inner Workings of Rhabdoid Tumors



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One Year Research Update: 

As a pediatric oncologist and investigator at the Memorial Sloan Kettering Cancer Center, Alex Kentsis, MD, PhD has a CureSearch Young Investigator grant to study the biology of rhabdiod tumors, one of the deadliest children's cancers. Specifically, Dr. Kentsis is interested in understanding the genetic make-up of these tumors, focusing on DNA sequences called transposons that can potentially move within cell genomes.

Dr. Kentsis previously found that in rhabdoid tumors, some transposons appear to be mobile and therefore have the potential to contribute to tumor growth and survival in response to chemotherapy.

Using a new DNA transposon mapping system, Dr. Kentsis analyzed DNA sequences of rhabdoid tumors and compared them to the normal genome. In doing so, he discovered thousands of mobile transposons. Now, he is investigating the role of these "mutant" transposons in the development of tumors in the laboratory.

He hopes that if he can successfully block tumor DNA transposition in animal models, this approach can eventually be translated into new therapies for children with rhabdoid tumors.



Alex Kentsis, MD, PhD, decided to study rhabdoid tumors because they remain one of the most lethal childhood cancers. Rhabdoid tumors affect mainly infants and young children and can be found in the kidneys, liver, soft tissue, and brain. Most children diagnosed with a rhabdoid tumor that cannot be completely removed through surgery do not have effective treatment options. As a pediatric oncologist and investigator at the Sloan-Kettering Cancer Center, Dr. Kentsis is working to understand the biology of this cancer, with a special emphasis on the genetic make-up of these tumors.

His research will focus on DNA sequences called transposons that can potentially move within cell genomes. Almost half of the human genome is made up of DNA derived from ancient transposons, but their activity in tumor cells is not understood. Researchers do know that when gone awry, they can potentially disrupt the normal workings of a cell. Dr. Kentsis has found that in rhabdoid tumors, some of these transposons appear to be mobile with potential contributions to tumor's growth and survival in response to chemotherapy. Understanding of mobile DNA in rhabdoid tumors could ultimately help to lead to new treatments.

 
  • Alex Kentsis, MD, PhD
  • Pediatric Oncologist / Investigator
  • Sloan-Kettering Cancer Center
 

Dr. Kentsis plans to use two approaches in his research. The first is to use specially developed methods to analyze the mobile DNA in the tumor genome to more fully understand the tumor's biology. He will then use this information to study human malignant rhabdoid tumors in genetically-engineered mice and zebrafish, both of which are effective models for studying human cancer. Dr. Kentsis hopes that if he can successfully block tumor DNA transposition in animal models, this approach can eventually be translated into new therapies for children with rhabdoid tumors. Dr. Kentsis also hopes that his study of mobile DNA in rhabdoid tumors will lead to a deeper understanding of genetics of other tumors.

His research will begin in 2013 and is funded by CureSearch for Children’s Cancer for two years through the Young Investigators Grant. During the course of his grant, Dr. Kentsis will provide updates on his research, and CureSearch looks forward to sharing those with you.


 

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