Presentation
Headache. History of multiple cerebral and cerebellar space occupying lesions discovered 2 years ago. Surgical excision of right cerebellar lesion. Now follow up.
Patient Data
Right cerebellar large recurrent space occupying lesion (SOL).
Multicenteric space occupying lesions scattered within both cerebral hemispheres (namely at the right parietal intraventricular region, septum pellucidum, both basal ganglia, right midbrain, right middle cerebellar peduncle, and left cerebellar hemisphere).
The lesions also involve the suprasellar cistern to involve the optic chiasm and the proximal optic nerve, likely via CSF dissemination..
The lesions elicit low signal on T1, high signal on T2 with small central cystic regions. The largest at the right parietal SOL shows thick wall and large necrotic center. Diffusion restriction of the right parietal and right cerebellar lesions.
Some of the lesions show nodular enhancement and others show marginal enhancement.
The large right intraventricular lesion is surrounded by vasogenic edema and causes entrapment of the right occipital and temporal horns
Excisional biopsy of the right cerebellar lesion
Case Discussion
You could initially associate NF1 with the left optic nerve and optic chiasm gliomas. Additionally, you might consider a pilocytic astrocytoma with piliod characteristics for the right parietal intraventricular aggressive-looking lesion, but given the patient's young age, this is unlikely. Also, the left optic nerve shows mild nodular post-contrast enhancement similar to the cerebral small lesions.
The patient underwent a surgical excisional biopsy of the right cerebellar lesion which revealed pleomorphic xanthoastrocytoma (PXA).
The patient had a stable appearance and size of the cerebellar lesions through 2 years follow-up period.
This is a rare case of synchronous multifocal PXA through both cerebral hemispheres.
Dissemination of the tumor cells via CSF pathways may explain the multicentric lesions as well as the optic chiasm and optic nerve involvement because the lesions were predominantly periventricular or adjacent to deep sulci.1
The multiplicity of PXA lesions increases the risk of anaplastic transformation.
This case is courtesy of Dr.Tamer Bakry
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