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A Rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior ­ Dysplastic Gangliocytoma of the Cerebellum: Case Report
Almeida, Leandro Pelegrini de; Schiavo, Felipe Lourezon; Santos, Samir Cezimbra dos; Nesi, William Mazzucco; Cambruzzi, Eduardo.
  • Almeida, Leandro Pelegrini de; Hospital Cristo Redentor. BR
  • Schiavo, Felipe Lourezon; Hospital Cristo Redentor. BO
  • Santos, Samir Cezimbra dos; Hospital Cristo Redentor. BR
  • Nesi, William Mazzucco; Hospital Cristo Redentor. BR
  • Cambruzzi, Eduardo; Santa Rita Hospital, Complexo Hospitalar Santa Casa. BR
Arq. bras. neurocir ; 39(2): 132-135, 15/06/2020.
Article in English | LILACS | ID: biblio-1362496
ABSTRACT
Dysplastic gangliocytoma of the cerebellum (DGC) or Lhermitte-Duclos Disease is a rare lesion (World Health Organization [WHO] grade I) characterized by thickened folia and replacement of the internal granular layer by abnormal ganglion cells. More commonly, the compromised patients are young adults presenting ataxia, seizures, obstructive hydrocephalus, and increased intracranial pressure. Dysplastic gangliocytoma of the cerebellum is intimately associated with Cowden syndrome, a hereditary disorder caused by a germline mutation in the PTEN tumor suppressor gene on chromosome 10q23. Large neurons of DCG show vesicular nuclei with prominent nucleoli. Expansion of the internal granular layer determines vacuolization of the molecular layer and white matter, which can be related to the bright stripes identified on T2-weighted magnetic resonance imaging. Herein, the authors report a female patient who developed long- time recurrence of DGC and discuss pathological findings and differential diagnosis of this rare cerebellar lesion.
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Full text: Available Index: LILACS (Americas) Main subject: Hamartoma Syndrome, Multiple / Cerebellar Neoplasms / Ganglioneuroma Type of study: Prognostic study Limits: Adult / Female / Humans Language: English Journal: Arq. bras. neurocir Journal subject: Cirurgia / Neurosurgery Year: 2020 Type: Article Affiliation country: Brazil Institution/Affiliation country: Hospital Cristo Redentor/BO / Hospital Cristo Redentor/BR / Santa Rita Hospital, Complexo Hospitalar Santa Casa/BR

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Full text: Available Index: LILACS (Americas) Main subject: Hamartoma Syndrome, Multiple / Cerebellar Neoplasms / Ganglioneuroma Type of study: Prognostic study Limits: Adult / Female / Humans Language: English Journal: Arq. bras. neurocir Journal subject: Cirurgia / Neurosurgery Year: 2020 Type: Article Affiliation country: Brazil Institution/Affiliation country: Hospital Cristo Redentor/BO / Hospital Cristo Redentor/BR / Santa Rita Hospital, Complexo Hospitalar Santa Casa/BR