title: Hemorrhagic presentations of cerebellar pilocytic astrocytomas in children resulting in death: report of 2 cases.
abstract: Acute hemorrhagic presentation in pilocytic astrocytomas (PAs) has become increasingly recognized. This type of presentation poses a clinically emergent situation in those hemorrhages arising in PAs of the cerebellum, the most frequent site, because of the limited capacity of the posterior fossa to compensate for mass effect, predisposing to rapid neurological deterioration. As examples, we describe two cases of fatal hemorrhagic cerebellar PAs: one of a child with a slowly growing stereotypical WHO Grade I PA with a 1-year period of symptomatology that preceded a rapid clinical deterioration, and another of an asymptomatic child having a PA variant, presenting with progressive obtundation following a presumed Valsalva event. These two scenarios parallel previous reports in the literature of either a setting of progressive expression of cerebellar dysfunction and transient episodes of raised intracranial pressure (ICP), or abrupt onset of features of increased ICP in a previously well child. The literature is further reviewed for a current understanding of the factors that predispose, initiate and propagate bleeding, with specific reference to the role of vascular endothelial growth factor and other angiogenic agents in the genesis and stability of the vasculature in PAs. In this context, we propose that obliterative vascular mural hyalinization with associated altered flow dynamics and microaneurysm formation was the pathogenesis of the hemorrhage in our first case. In the second case, large tumor size, increased growth rate, looseness of the background myxoid matrix, and thinness of the tumor blood vessels with calcospherite deposition predisposed to vascular leakage and bleeding concurrent with sudden increases in intravascular hydrostatic pressure. In that cerebellar PAs are common, this report underscores the importance of considering in the differential diagnosis the possibility of a spontaneous hemorrhage in a posterior fossa PA in a child presenting with a sudden neurological ictus and raised ICP.
authors.affiliation: Departments of 1 Radiology and Diagnostic Imaging.
authors.forename: Mitchell P
authors.initials: MP
authors.lastname: Wilson
authors.affiliation: Laboratory Medicine and Pathology.
authors.forename: Edward S
authors.initials: ES
authors.lastname: Johnson
authors.affiliation: Division of Pathology and Labatt Brain Tumor Research Centre, Hospital for Sick Children, University of Toronto, Ontario, Canada; and.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Hawkins
authors.affiliation: Faculty of Medicine and Dentistry, and.
authors.forename: Kerry
authors.initials: K
authors.lastname: Atkins
authors.affiliation: Division of Neurosurgery, Departments of Surgery and Clinical Neurosciences, University of Alberta, Edmonton, Alberta;|King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia.
authors.forename: Wael
authors.initials: W
authors.lastname: Alshaya
authors.affiliation: Division of Neurosurgery, Departments of Surgery and Clinical Neurosciences, University of Alberta, Edmonton, Alberta;
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Pugh
mesh_terms: D001254:Astrocytoma / Q000150:complications / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000150:complications / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D020300:Intracranial Hemorrhages / Q000209:etiology / Q000473:pathology*; D008297:Male
keywords: BRAF; EVD = external ventricular drain; GCS = Glasgow Coma Scale; ICP = intracranial pressure; PA = pilocytic astrocytoma; PCR = polymerase chain reaction; VEGF; VEGF = vascular endothelial growth factor; hemorrhage; juvenile pilocytic astrocytoma; low-grade astrocytoma; pediatric; pilocytic astrocytoma; vascular disorders