161 Pediatric Versus Adult Pilocytic Astrocytoma (PA): Largest Comparison Cohort of Clinical Course & Genomic Profile Demonstrates BRAF-KIAA1549 Fusion is Associated With Worse Prognosis in Children Compared to Adults

INTRODUCTION: Recent work demonstrates significant associations between genomic status and clinical outcomes in pediatric pilocytic astrocytoma (PA). To date, a detailed comparison with adult PA patients has yet to be made. METHODS: We retrospectively reviewed treatment for pediatric and adult PA (2000-2023). BRAF-KIAA1549(B-K) fusion and BRAF-V600E mutations detected through immunohistochemistry, chromosomal-microarray, and next-generation sequencing were identified via pathology records. RESULTS: We identified 315 children (mean age at diagnosis 8.1 years, 50.8% female) and 29 adults (34.5 years, 41.4% female). Most common tumor locations were posterior fossa (59.7% pediatric, 51.7% adult) and suprasellar (21.3% pediatric, 24.1% adult). Pediatrics were more likely to experience obstructive hydrocephalus (p<0.01) necessitating EVD placement (p=0.02). Gross-total resection was achieved in 59.4% pediatric and 48.3% adults (p=0.08). Median length of postoperative stay was shorter in adults (4 versus 8 days, p=0.002). Adjuvant chemotherapy was more common in children (26% versus 3.4%, p=0.013), while adults were more likely to receive adjuvant radiotherapy (27.6% versus 1.3%, p< 0.001). BRAF-V600E mutations were identified in 8/67 children and 1/9 adults (p=0.48); B-K fusions were identified in 44/76 (57.9%) children and 2/10(20%) adults tested (p=0.055). Tumor progression/recurrence occurred in 5 (17.2%) adults and 69(21.9%) children (p=0.65). Median progression/recurrence-free survival (PRFS) time for children and adults was 4.6 and 2.2 years respectively (p=0.81, log-rank test). BRAF-V600E mutation did not confer worse PRFS compared to wildtype in either population. B-K fusion was associated with significantly shorter PRFS in pediatrics (3.0 versus 5.4 years, p=0.036), a trend which was not statistically significant in adults (p=0.2). CONCLUSIONS: This represents the largest cohort comparing genetics/clinical course of pediatric versus adult PA. Rates of obstructive hydrocephalus and adjuvant chemotherapy use were more common in children; radiotherapy was favored for adults. B-K fusions were more frequent in pediatrics and associated with worse PRFS than adults.

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Publication Details

Journal
Neurosurgery
Published
2025-03-14
DOI
https://doi.org/10.1227/neu.0000000000003360_161
Primary Topic
Glioma Diagnosis and Treatment
Type
article
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article

161 Pediatric Versus Adult Pilocytic Astrocytoma (PA): Largest Comparison Cohort of Clinical Course & Genomic Profile Demonstrates BRAF-KIAA1549 Fusion is Associated With Worse Prognosis in Children Compared to Adults

Shuting Mao, Arman Jahangiri, Kimberly Bojanowski Hoang, Joshua J. Chern et al.
Neurosurgery
Glioma Diagnosis and Treatment
article

161 Pediatric Versus Adult Pilocytic Astrocytoma (PA): Largest Comparison Cohort of Clinical Course & Genomic Profile Demonstrates BRAF-KIAA1549 Fusion is Associated With Worse Prognosis in Children Compared to Adults

Shuting Mao, Arman Jahangiri, Kimberly Bojanowski Hoang, Joshua J. Chern, Katherine Chandler, Tianwen Ma, Julia Grigorian, Jocelyn Chow, Zvipo Chisango, Vivek A. Pisharody
article en

Abstract

INTRODUCTION: Recent work demonstrates significant associations between genomic status and clinical outcomes in pediatric pilocytic astrocytoma (PA). To date, a detailed comparison with adult PA patients has yet to be made. METHODS: We retrospectively reviewed treatment for pediatric and adult PA (2000-2023). BRAF-KIAA1549(B-K) fusion and BRAF-V600E mutations detected through immunohistochemistry, chromosomal-microarray, and next-generation sequencing were identified via pathology records. RESULTS: We identified 315 children (mean age at diagnosis 8.1 years, 50.8% female) and 29 adults (34.5 years, 41.4% female). Most common tumor locations were posterior fossa (59.7% pediatric, 51.7% adult) and suprasellar (21.3% pediatric, 24.1% adult). Pediatrics were more likely to experience obstructive hydrocephalus (p<0.01) necessitating EVD placement (p=0.02). Gross-total resection was achieved in 59.4% pediatric and 48.3% adults (p=0.08). Median length of postoperative stay was shorter in adults (4 versus 8 days, p=0.002). Adjuvant chemotherapy was more common in children (26% versus 3.4%, p=0.013), while adults were more likely to receive adjuvant radiotherapy (27.6% versus 1.3%, p< 0.001). BRAF-V600E mutations were identified in 8/67 children and 1/9 adults (p=0.48); B-K fusions were identified in 44/76 (57.9%) children and 2/10(20%) adults tested (p=0.055). Tumor progression/recurrence occurred in 5 (17.2%) adults and 69(21.9%) children (p=0.65). Median progression/recurrence-free survival (PRFS) time for children and adults was 4.6 and 2.2 years respectively (p=0.81, log-rank test). BRAF-V600E mutation did not confer worse PRFS compared to wildtype in either population. B-K fusion was associated with significantly shorter PRFS in pediatrics (3.0 versus 5.4 years, p=0.036), a trend which was not statistically significant in adults (p=0.2). CONCLUSIONS: This represents the largest cohort comparing genetics/clinical course of pediatric versus adult PA. Rates of obstructive hydrocephalus and adjuvant chemotherapy use were more common in children; radiotherapy was favored for adults. B-K fusions were more frequent in pediatrics and associated with worse PRFS than adults.

NeurosurgeryVol. 71(Supplement_1)
Openalex Percentile: Top 93%
Glioma Diagnosis and Treatment
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