Cerebellar gangliocytoma

Case contributed by Dr Wen Jak Ma

Presentation

Right sensorineural hearing loss

Patient Data

Age: 70 years
Gender: Male
MRI

Deep right inferior cerebellar mass. It is T1 hypointense, heterogeneous on T2 with intermediate and bright signal, but more homogeneously bright on FLAIR. Equivocal foci of diffusion restriction not corresponding to any particularly bright or dark T2 areas. Patchy internal nodular enhancement, not corresponding to areas of diffusion restriction. On SWI, there are a few tiny anomalous vessels throughout the mass.

Pathology

Increased number of ganglion cells that show large nuclei, prominent nucleoli, and abundant cytoplasm. Some nuclei show marked enlargement and binucleated forms are also seen.

These atypical ganglion cells are embedded within delicate neuropil. Diagnosis: gangliocytoma (WHO Grade 1).

No neoplastic astrocytic component to suggest a ganglioglioma.

Case Discussion

A rare case of cerebellar gangliocytoma, without dysplastic cerebellar tissue or neoplastic glial component. This is similar to the supratentorial gangliocytoma in young patients and is a different entity from the dysplastic cerebellar gangliocytoma aka Lhermitte-Duclos disease.

Tiny anomalous vessels or neovascularity in the mass is a peculiar finding.

Very late (and incidental) presentation at 70 years old!

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Case information

rID: 65377
Published: 11th Jan 2019
Last edited: 25th Jan 2019
Inclusion in quiz mode: Included

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