Primitive neuroectodermal tumour of the CNS

Case contributed by Dr Prashant Mudgal

Presentation

Patient presented with altered mental status

Patient Data

Age: 9 years
Gender: Female

A large well defined heterogeneously enhancing intra-axial solid cystic lesion is noted in the right fronto-temporo-parietal region, and basal ganglia with surrounding vasogenic edema. The lesion is heterogeneously hypointense on T1, hyperintense on T2 and isointense on FLAIR. The lesion shows few areas of blooming on SWI (not available) and restriction on DWI.

The lesion shows non enhancing cystic nacrotic areas within with few areas of T1 hyperintensities suggesting haemorrhage.

Medially the lesion is exerting the mass effect causing compression of right lateral ventricle and displacement of midline 1.4 cm towards the left. There is obstructive dilatation of left lateral ventricle and third ventricle with periventricular ooze.

Inferomedially the lesion is compressing and displacing the midbrain towards contralateral side. It is encasing and displacing the right MCA and displacing the right ACA.

Inferiorly it is abutting the posterior clinoid process sparing the sella and optic chiasma.

Anteroinferiorly it is extending upto the sphenoid wing, however there is no calvarial erosion noted. Posterior extension of the lesion is up to the tentorium.

MR spectroscopy was done later( not available) showed significantly elevated choline, reduced NAA and creatine and elevated lipid/lactate within the lesion with choline/creatine ratio of 3.2.

Morphology of the lesion and MRS findings are suggestive of high grade tumour most likely PNET

 

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Case information

rID: 32696
Case created: 9th Dec 2014
Last edited: 15th Jan 2017
Tag: pm
Inclusion in quiz mode: Included

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