Diffuse paediatric cerebellar glioma: two identical imaging phenotypes of an extremely rare entity with disparate pathology

Childs Nerv Syst. 2023 Apr;39(4):857-861. doi: 10.1007/s00381-023-05836-0. Epub 2023 Jan 20.

Abstract

Although the posterior fossa is a common location for paediatric brain tumours [1], diffuse glioma isolated to the cerebellum is an extremely rare imaging entity. Only two cases of isolated diffuse paediatric cerebellar glioma have been reported in the English language to the best of our knowledge [2, 3], and only one of these cases had a similar imaging phenotype to our cases [3]. Although somewhat similar to Lhermitte-Duclos (dysplastic gangliocytoma of the cerebellum), the appearances are distinct from other neoplastic entities of the paediatric posterior fossa. Clinical presentation and neurological examination findings are vital however to help differentiate other diffuse pathologies involving the cerebellum such as rhombencephalitis. Presented here are two diffuse cerebellar gliomas in children under the age of 3 with near identical imaging phenotypes demonstrating differing histological and molecular genetic profiles.

Keywords: Diffuse cerebellar glioma paediatrics.

Publication types

  • Review

MeSH terms

  • Brain Neoplasms* / pathology
  • Cerebellar Neoplasms* / diagnostic imaging
  • Cerebellar Neoplasms* / genetics
  • Cerebellum / diagnostic imaging
  • Glioma* / diagnostic imaging
  • Glioma* / genetics
  • Glioma* / pathology
  • Hamartoma Syndrome, Multiple* / genetics
  • Hamartoma Syndrome, Multiple* / pathology
  • Hamartoma Syndrome, Multiple* / surgery
  • Humans
  • Magnetic Resonance Imaging