title: Rapid and fulminant leptomeningeal spread following radiotherapy in diffuse intrinsic pontine glioma.
abstract: A 4-year-old male presented with rapid-onset cranial nerve palsy and ataxia. Brain magnetic resonance imaging (MRI) revealed a pontine mass lesion with discordant conventional and advanced imaging. A stereotactic core biopsy revealed glioblastoma with immunostaining suggestive of histone H3K27M and TP53 mutation, consistent with diffuse intrinsic pontine glioma. MRI 3 months after radiotherapy revealed extensive new leptomeningeal metastatic disease involving both the supra- and infratentorial brain, as well as the imaged portion of the spine. Tissue procured at the time of needle biopsy has undergone striking in vivo expansion as an orthotopic xenograft.
authors.affiliation: Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Tinkle
authors.affiliation: Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Brent A
authors.initials: BA
authors.lastname: Orr
authors.affiliation: Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: John T
authors.initials: JT
authors.lastname: Lucas
authors.affiliation: Department of Neurosurgery, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Paul
authors.initials: P
authors.lastname: Klimo
authors.affiliation: Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Zoltan
authors.initials: Z
authors.lastname: Patay
authors.affiliation: Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Suzanne J
authors.initials: SJ
authors.lastname: Baker
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Alberto
authors.initials: A
authors.lastname: Broniscer
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Qaddoumi
mesh_terms: D020295:Brain Stem Neoplasms / Q000473:pathology* / Q000532:radiotherapy*; D002675:Child, Preschool; D018450:Disease Progression; D017809:Fatal Outcome; D005909:Glioblastoma / Q000473:pathology* / Q000532:radiotherapy*; D006801:Humans; D008297:Male; D055756:Meningeal Carcinomatosis / Q000473:pathology*
keywords: H3K27M; hypofractionated radiotherapy; leptomeningeal metastasis; pontine glioma; stereotactic biopsy; xenograft