Atypical medulloblastoma

Case contributed by Dr Ian Bickle

Presentation

18 month old. Signs of truncal ataxia.

Patient Data

Age: 1
Gender: Male
MRI

Gross asymmetry of the cerebellar hemispheres.

Markely thickened folia in the right cerebellar hemisphere, causing mass effect on the vermis, brainstem and pons, with an element of cerebellar tonsillar descent.

Displacement and compression of the 4th ventricule with a resultant severe biventricular hydrocephalus.

Transependymal oedema.

Multisepatated cystic component on the medial aspect.

CT

Neurosurgery was performed overseas

Right posterior fossa craniectomy.

Resection of the right cerebeller mass.  No recurrent or residual disease.

Case Discussion

This case was initially thought to represent Lhermitte-Duclos disease (LDD) also termed dysplastic cerebellar gangliocytoma, is a rare tumour of cerebellum.  Multiple radiologists with varying degrees of sub-speciality on 3 continents thought this is the most likely diagnosis.

In Lhermitte-Duclos the abnormal tissue involves the cerebellar cortex, and is usually confined to one hemisphere.  It can atypically, as in this case, have a cystic component. Enhancement is not a typically a feature, although in the case mild enhancement is present. The dysplastic ''mass'' grows slowly with patient presentation usually related to the symptomatology resulting from obstructive hydrocephalus. The extent of the mass varies, with this case being particularly profound such the degree of the resultant mass effect.

The chief differential for the appearances in this age group is an atypical medulloblastoma - one with extensive nodularity.

Surgical resection was undertaken, at which time the surgeon stated it didnt seem at all like a medulloblastoma.

It was histopathological confirmation was of a medulloblastoma.

 

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Case information

rID: 34903
Case created: 13th Mar 2015
Last edited: 5th May 2017
Inclusion in quiz mode: Included

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