ArticleBrain & spine2026
Intracranial hemangioblastomas in children: Clinical-radiological characteristics, microsurgical strategy, and long-term outcomes in a 10-year single-center cohort.
Article in Brain & spine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: Intracranial hemangioblastomas (HBs) are exceedingly rare in the pediatric population, and their clinical-radiological features, optimal treatment modalities, and long-term prognosis remain inadequately defined. Research question: We sought to characterize the clinical-radiological characteristics, treatment modalities and clarify the surgical outcomes for pediatric patients with brain HBs. Material and methods: Consecutive patients aged ≤18 years who underwent resection for intracranial HBs between 2011 and 2021 were retrospectively reviewed. Epidemiologic, clinical, neuroimaging, treatment modalities, and follow-up data were integrated and reviewed. Results: Thirty-three patients (male:female = 1.2:1; mean age 14.9 ± 2.8 years) were identified. Eighteen cases (54.5%) were sporadic, 15 (45.5%) were von Hippel-Lindau (VHL)-associated. Presenting manifestations mainly reflected posterior-fossa hypertension and cerebellar dysfunction: headache (n = 21, 63.6%), vomiting (n = 13, 39.4%), and gait instability (n = 9, 27.3%). Neuroimaging revealed a predominant cerebellar location (n = 30, 76.9%); most (n = 29, 74.4%) exhibited the classic cyst-with-enhancing-nodule architecture. VHL-associated HBs were smaller (p = 0.039) and more frequently multi-focal. Gross-total resection (GTR) achieved in 94.9% lesions. At follow-up, all recurrences (n = 4, 12.1%) occurred in the VHL cohort, yielding a 5-year progression-free survival (PFS) of 87.9% for the entire series but only 73.3% for VHL patients. Multi-focal central nervous system (CNS) and extra-CNS VHL lesions necessitated additional interventions in 53.3% of VHL cases. Discussion and conclusion: Pediatric intracranial HBs demonstrate unique features, including a higher rate of VHL association. Early surgical intervention remains the main treatment methods, ensuring favorable outcomes when performed properly. Life-long follow-up is essential, particularly for those with VHL-associated tumors, due to their higher recurrence risk.
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