Pleomorphic xanthoastrocytoma

Case contributed by Dr Vikash Kumar Gupta

Presentation

Presented with focal seizures. Had earlier episode of seizures in childhood

Patient Data

Age: 14 years
Gender: Male
Modality: MRI

Relatively well-marginated mass lesion in right temporo-parietal lobe showing heterogeneously hyperintense signal on T2W images with hypo to intermediate signal on T1W images. 

Case Discussion

Pleomorphic Xanthoastrocytomas (PXAs) account for less than 1 % of all astrocytomas and are classified as grade II tumours by WHO. They are usually seen in the second decade of life with a mean age at the time of presentation being 26 years.  More than 99% of these tumours are supratentorial, located superficially in cerebral hemispheres, with a predilection for the temporal and frontal lobes.

Patients usually present with seizures considering the tumour predilection for the temporal lobes. The seizures are generally long-standing (mean 7.6 years).

On MRI these tumours appear as complex solid-cystic masses with solid portion avidly enhancing both on CT and MRI.  The cystic component usually lies deeper to solid component. Usually, PXAs are not associated with significant perilesional oedema. 

This case did not show typical MR features of PXAs, and therefore we gave the differential of ganglioglioma and DNET as well. Histopathological examination together with IHC confirmed pleomorphic xanthoastrocytoma.

PXAs can recur and show aggressive behaviour with a mortality rate of between 15% to 20%. However, these tumours have a relatively favourable outcome with overall survival rates of 75%– 81% at five years and 67%– 70% at ten years.

Common differentials are desmoplastic infantile gliomas (seen in infants) and DNET (usually shows gyriform configuration).  

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Case Information

rID: 51175
Case created: 6th Feb 2017
Last edited: 4th Apr 2017
Inclusion in quiz mode: Included

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