Hemorrhagic presentations of cerebellar pilocytic astrocytomas in children resulting in death: report of 2 cases
| Autor: | Cynthia Hawkins, Kerry Atkins, Mitchell P Wilson, Jeffrey A. Pugh, Edward S Johnson, Wael Alshaya |
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| Rok vydání: | 2016 |
| Předmět: |
Male
Pathology medicine.medical_specialty Obtundation Hydrostatic pressure Context (language use) Astrocytoma 03 medical and health sciences Fatal Outcome 0302 clinical medicine Humans Medicine Cerebellar Neoplasms Child Pilocytic astrocytoma business.industry Cerebellar Neoplasm Juvenile Pilocytic Astrocytoma General Medicine medicine.disease Child Preschool 030220 oncology & carcinogenesis Female Differential diagnosis business Intracranial Hemorrhages 030217 neurology & neurosurgery |
| Zdroj: | Journal of Neurosurgery: Pediatrics. 17:446-452 |
| ISSN: | 1933-0715 1933-0707 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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