Case Presentation: Systemic Second-Line Treatment for BRAF-Altered Pediatric Low-Grade Glioma Case Presentation
Patient Case:
A 6-year-old male patient was discovered to have a posterior fossa mass and obstructive hydrocephalus on MR imaging after presenting to the emergency room with progressive headaches for one month and associated nausea and vomiting.
After an emergent external ventricular drain was placed to relieve his hydrocephalus, the patient underwent a craniotomy of the mass. The mass was determined to be a pilocytic astrocytoma WHO Grade 1 by histological assessment from a sub-total resection. The tumor was found to be BRAF V600E mutated on molecular profiling, and systemic therapy was initiated with Dabrafenib and Trametinib as first-line treatment for residual, unresectable tumor.
After 18 months of treatment, the tumor showed consistent progression in volume and enhancement on 2 consecutive imaging intervals. Because surgical resection posed significant risk to the patient, a stereotactic biopsy was performed to assess for malignant transformation.
Histological and molecular evaluation were consistent with the initial diagnosis without identifiable changes. He was thus considered for systemic second-line treatment of his progressing low-grade glioma, by selection of a treatment modalities that is FDA-approved for children with BRAF-altered recurrent or refractory low-grade glioma, and proven effective for tumor control as second-line to initial combination treatment with dabrafenib and trametinib.
