Abstract #5

Presenter

Adrian Levine completed his medical school at the University of Western Ontario (2016) and neuropathology residency at the University of British Columbia (2021). He is currently in the Clinician Investigator Program, doing a research fellowship at the Hospital for Sick Children in Toronto, under the supervision of Dr Cynthia Hawkins. His clinical interests include tumor neuropathology and molecular pathology, and research interests include cancer genetics and bioinformatics/machine learning.

Authors

Adrian Levine,^1,4 Julie Bennett, ² Joseline Haizel-Cobbina, ³ Liana Nobre,² Michael Dewan,³ Uri Tabori,² and Cynthia Hawkins¹

1. Department of Pediatric Laboratory Medicine, Hospital for Sick Children, Toronto, ON
2. Division of Neuro-Oncology, Hospital for Sick Children, Toronto, ON
3. Department of Neurosurgery, Vanderbilt University Medical Center, Nashville TN
4. Clinician Investigator Program, University of British Columbia, BC

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The international disseminated pediatric low grade glioma consortium: project goals and preliminary results

Abstract

Although most pediatric LGG (PLGG) have excellent long-term survival, there is a subset of cases that disseminate (DPLGG) and have very poor outcomes. It is unknown why these tumors behave in such an aggressive way. We believe that biological mechanisms, distinct from those present in non-disseminated LGG, underlie this metastatic ability, but so far there has been no in-depth investigation of this.

 To improve our understanding of this rare patient population, we have created a new consortium to compile DLGG cases across from across the world. We are collecting comprehensive clinical information, including the tumor presentation and pattern of dissemination, surgical outcomes and subsequent treatment courses, and quality of life outcomes. Cases with available tissue will be tested with our glioma NGS panel for the most common mutations and fusions, and methylation array for copy number and classification.

 Our data thus far on the first 53 cases demonstrates the DPLGG has much worse prognosis than the overall PLGG population. Virtually all DPLGG progress at 5 years, compared to a quarter of other PLGG, and the disseminated cases are approximately 5-times more likely to die at 10 years. This can affect children of all ages and does not carry a gender predilection. We observe three patterns of dissemination – 40% of patients present with a localized mass and have secondary dissemination, 35% with disseminated tumor and a clear dominant mass, and 25% with disseminated disease without a dominant mass. The most common molecular alteration is BRAF fusions.