Evaluation of MYBL1 Fusion Oncogene in Pediatric Diffuse Astrocytoma

Pediatric diffuse astrocytomas are rare but represent a major clinical problem in pediatric neuro-oncology due to their heterogeneous pathology and unpredictable clinical behavior. Unlike adult gliomas researchers’ understanding of the molecular mechanisms which drive tumorigenesis within pediatric low grade gliomas are largely unknown. Recent genomic studies by the Dana Farber Cancer Institute identified several new copy number aberrations which appear to represent the first definitions of new subclasses within this disease which may be useful for targeting therapy or predicting tumor behavior.

Principal Investigator Dr. Keith Ligon proposed to further accelerate understanding the biology of normal MYBL1 andMYBL1tr in brain development and pediatric disuse astrocytomas while also establishing MYBL1tr as a biomarker, seeking to do this through two specific aims: construction of a transgenic mouse model expressing MYBL1tr in the brain and evaluation of the outcomes of patients found to have MYBL1 using IHC and bioinformatics approaches.

Funded in 2013.