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Research Highlight | December 14, 2023

BC team identifies drivers of aggressive childhood cancers and develops model to explore new treatments

In a recent study funded by The Terry Fox Research Institute, a team of researchers in British Columbia verified two drivers of cancerous tumours caused by DICER1 syndrome and developed an animal model that will allow them to track DICER1 cancer growth and test novel therapies.

The syndrome is a rare genetic disorder mainly affecting children that increases the risk of cancers impacting multiple organs.

For patients with DICER1 syndrome, cancerous and non-cancerous tumours are caused by a mutation in the DICER1 gene, which regulates the function of other genes. In most cases, this mutation is passed down from a parent, though it can also occur due to new mutations. Often the disease lies dormant unless certain secondary mutations drive tumours to become aggressive and life-threatening.

“There is no effective treatment for these cancers,” says Dr. David Huntsman, distinguished scientist at BC Cancer Research Institute and lead author of the study published in Cancer Research.

“While we discovered the genetic drivers of these tumours a decade ago, no progress has been made in understanding the biology of cancer development and improving the disease treatment. This is due to a lack of scientific models for the disease.”

Funding from TFRI has changed that.

“We have created a first-ever mouse model that faithfully reflects the gynecologic tumour development in DICER1 syndrome patients, opening up opportunities to advance the research by developing models to capture the spectrum of tumours in this syndrome and to develop therapeutics,” says Dr. Yemin Wang, staff scientist at BC Cancer Research Institute and co-lead author of the study.

Moving forward, Drs. Huntsman and Wang and their team will continue using these models and derivative cell lines to explore novel therapies that can be put to clinical trials in hopes of improving the outcome for patients with DICER1-associated cancers.

“TFRI funding is absolutely essential for us, through supporting students, technicians and research expenses to develop and characterize this novel animal model,” says Dr. Wang.

This study was funded by a Terry Fox New Frontiers Program Project Grant in New vistas on cancer biology and treatments: conceptual advancements from the forme fruste project led by Dr. David Huntsman.