• Alexandre Roux, Nathalie Boddaert, Jacques Grill, David Castel, Marc Zanello, Gilles Zah-Bi, Fabrice Chrétien, Etienne Lefevre, Volodia Dangouloff Ros, Michel Zerah, Stéphanie Puget, Johan Pallud, and Pascale Varlet.
• Department of Neurosurgery, Sainte-Anne Hospital, Paris, France.
• Neurosurgery. 2020 Apr 1; 86 (4): 517-523.

BackgroundNo link has been demonstrated between diffuse intrinsic pontine glioma and developmental venous anomaly in pediatric patients.ObjectiveTo determine the prevalence of developmental venous anomaly in a pediatric cohort of diffuse intrinsic pontine glioma.MethodsWe performed a retrospective cohort study (1998-2017) of consecutive pediatric patients harboring a diffuse intrinsic pontine glioma (experimental set, n = 162) or a craniopharyngioma (control set, n = 142) in a tertiary pediatric neurosurgical center. The inclusion criteria were the following: age <18 yr at diagnosis; histopathological diagnosis of diffuse intrinsic pontine glioma or craniopharyngioma according to the 2016 World Health Organization classification of tumors of the central nervous system; no previous oncological treatment; and available preoperative magnetic resonance imaging performed with similar acquisition protocol.ResultsWe found a significantly higher prevalence of developmental venous anomaly in the experimental set of 162 diffuse intrinsic pontine gliomas (24.1%) than in the control set of 142 craniopharyngiomas (10.6%; P = .001). The prevalence of developmental venous anomalies was not significantly impacted by demographic data (sex, age at diagnosis, and underlying pathological condition), biomolecular analysis (H3-K27M-mutant subgroup, H3.1-K27M-mutant subgroup, and H3.3-K27M-mutant subgroup), or imaging findings (anatomic location, anatomic extension, side, and obstructive hydrocephalus) of the studied diffuse intrinsic pontine gliomas.ConclusionWe report a higher prevalence of developmental venous anomaly in pediatric diffuse intrinsic pontine glioma patients than in control patients, which suggests a potential underlying common predisposition or a causal relationship that will require deeper investigations.Copyright © 2019 by the Congress of Neurological Surgeons.

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