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A Case Report of Infant-Type Hemispheric Glioma with a Novel GAB1-ABL2 Kinase Fusion Treated with Dasatinib
Emery Buckner-Wolfson1, Geena Jung1, Timothy Kim1
1Department of Neurosurgery, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx, New York, USA.
Insights
A novel gene fusion in infant-type hemispheric glioma (IHG) was identified and treated with dasatinib. This targeted therapy showed significant tumor reduction, offering a new treatment avenue for this rare pediatric cancer.
Area of Science:
- Pediatric Oncology
- Molecular Diagnostics
- Genetics
Background:
- Infant-type hemispheric glioma (IHG) is a rare pediatric high-grade glioma.
- IHG commonly presents with receptor tyrosine kinase (RTK) gene fusions.
- Novel gene fusions in IHG present diagnostic and therapeutic challenges.
Introduction:
Infant-type hemispheric glioma (IHG) is a rare form of cancer that affects newborns and infants. It is classified as a pediatric-type high-grade glioma and typically harbors receptor tyrosine kinase (RTK) gene fusions. Here, we present the finding of a novel gene fusion IHG treated with a targeted therapy that has yet to be implemented for any other IHG case to date.
Case Presentation:
We report the case of a 12-month-old boy with IHG who presented with obstructive hydrocephalus due to a large mass in the right frontal lobe. The patient initially underwent mass resection, but subsequent imaging showed rapid interval progression of the residual tumor. Comprehensive molecular analysis of the tumor tissue revealed a novel GAB1-ABL2 gene fusion, and the patient was started on dasatinib, an ABL kinase inhibitor. Shortly after initiation of dasatinib treatment, there was a significant reduction in tumor size and enhancement, followed by stabilization of disease.
Discussion:
The patient's robust response to treatment suggests that dasatinib is an effective targeted therapy for IHG harboring a GAB1-ABL2 gene fusion. This finding may inform future investigations into the disease processes of IHG and help guide the diagnosis and treatment of IHG in the absence of previously identified gene fusions, improving clinical management of this vulnerable patient population.
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