A Rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior Dysplastic Gangliocytoma of the Cerebellum: Case Report
Arq. bras. neurocir
; 39(2): 132-135, 15/06/2020.
Article
em En
| LILACS
| ID: biblio-1362496
Biblioteca responsável:
BR1.1
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Assunto principal:
Síndrome do Hamartoma Múltiplo
/
Neoplasias Cerebelares
/
Ganglioneuroma
Tipo de estudo:
Prognostic_studies
Limite:
Adult
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Female
/
Humans
Idioma:
En
Revista:
Arq. bras. neurocir
Assunto da revista:
Cirurgia
/
NEUROCIRURGIA
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Brasil
País de publicação:
Brasil