Dysplastic cerebellar gangliocytoma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Concern for multiple sclerosis. Oligoclonal bands on CSF analysis.

Patient Data

Age: 60 years
Gender: Female

There is an abnormal, mass-like T2 FLAIR hyperintense signal abnormality involving the vermis and superior left cerebellar hemisphere (partially extending into the left brachium pontis) associated with mass effect upon the fourth ventricle.

Although the mass has high signal on the b=1000 images, it has slightly facilitated diffusion on ADC compared to adjacent normal brain parenchyma (thus T2 shine through).
 
Scattered foci of T2 FLAIR hyperintensity involving the periventricular and subcortical white matter are non-specific.

Case Discussion

The patient went on to have a biopsy/resection.

Histology

Surgical resection of the mass revealed scattered large abnormal ganglion cells in the white matter underneath the internal granule cell layer, with no mitotic activity, necrosis or microvascular proliferation.

Immunohistochemistry

  • Neu-N: positive

  • GFAP and neurofilament: weak staining

  • Ki-67: <1%

  • There was no significant reticulin production by the tumor.

Diagnosis

Along with the radiologic findings, these findings are consistent with a dysplastic cerebellar gangliocytoma (L'hermitte Duclos).

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