Intraventricular Ectopic Cerebellum

Alessandro De Benedictis, Maria Camilla Rossi-Espagnet, Francesca Diomedi-Camassei, Sabrina Rossi, Elena Fontana, Franco Randi, Viviana Ponzo, Carlotta Nucci, Giacomo Esposito, Giovanni Paternò, Carolina Brunetti, Alessandra Savioli, Andrea Carai, Carlo Efisio Marras

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Cerebellar ectopy is a rare finding, with few cases previously reported. Intraventricular localized cerebellar ectopy was described in only 1 case within the fourth ventricle. Case Description: A 9-year-old girl suffered for 2 years from bilateral frontoparietal headaches, sometimes accompanied by vomiting and photophobia. Magnetic resonance imaging demonstrated an oval-shaped lesion within the left lateral ventricle, characterized by well-defined margins without a clear cleavage plane from the adjacent choroid plexus. The mass presented an intermediate signal on T1- and T2-weighted sequences, similar to gray matter, and reduced ADC values on ADC maps compared with white matter, with no enhancement after gadolinium-based contrast injection. After resection, macroscopic examination revealed an organoid structure with leptomeningeal lining and a clear-cut cortex and white matter components. Histology demonstrated normal cerebellum with a double-layered cortex and normal underlying white matter. The cerebellar ectopy was focally covered by bundles of capillary vascular structures covered by a monostratified ependymal cell lining, consistent with choroid plexus. Conclusions: We describe, for the first time to our knowledge, the case of a child with ectopic cerebellar tissue harboring the supratentorial ventricular system. Plausible etiologic mechanism consists in the herniation of the cerebellar germinal tissue into the ventricular system through the ependyma, allowing cell migration to the supratentorial compartment, followed by maturation into the normal cerebellum.

Original languageEnglish
Pages (from-to)158-163
Number of pages6
JournalWorld Neurosurgery
Volume137
DOIs
Publication statusPublished - May 2020

Keywords

  • Abnormal location
  • Child
  • Ectopic cerebellum
  • Lateral ventricle
  • Neurosurgery

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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