Brigatinib in ALK-fused, Low-grade Pediatric Angiocentric Glioma with Multiple Extracranial Metastases: A Case Report.

BACKGROUND

Gliomas are the most frequent Central Nervous System (CNS) tumors in children and adolescents. Angiocentric glioma is a rare subtype of pediatric-type diffuse low-- grade gliomas with a relatively favorable prognosis following gross total resection. More recently, the identification of Anaplastic Lymphoma Kinase (ALK) fusions in glioma has led to the development of targeted therapeutics to improve responses and outcomes.

CASE PRESENTATION: Here, we report a 10-year-old boy with pediatric low-grade glioma harboring a KIF5B-ALK fusion. He achieved a durable partial response to the ALK inhibitor brigatinib. The patient presented with nasal congestion, dizziness, and headache. MRI and CT scans revealed multiple masses in the brain, lung, and bone. Pathological review indicated a low-grade glioma, and genetic testing identified a KIF5B-ALK fusion. After surgical resection and molecular diagnosis, the patient was treated with brigatinib. This resulted in rapid tumor reduction and complete remission at 23 months, with no significant adverse effects.

CONCLUSION

This case highlights the importance of molecular profiling in guiding targeted therapies for rare pediatric gliomas. Further research is needed to address questions regarding the optimal use of ALK inhibitors and their long-term impact on prognosis and neurocognitive development.