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Pleomorphic Xanthoastrocytoma Arising from Olfactory Groove: A Rare Location for a Rare Tumor

Pleomorphic xanthoastrocytomas (PXAs) are a rare variety of WHO grade II gliomas in children and young adults. The common site of location of PXAs is temporal or cortical. We present a rare case of PXA presenting as anterior cranial fossa (ACF) base lesion. A 7-year-old male presented with a history of a single episode of generalized tonic-clonic seizures and anosmia of 1 year's duration. An MRI of the brain revealed a large solid and cystic tumor in the ACF base with leptomeningeal involvement. The origin of the tumor could not be made out. The patient underwent parasagittal frontal craniotomy and gross total resection of the tumor. The tumor was parenchymal, involving both frontal lobes. It seemed to have been arising from the olfactory groove region. The solid portion of the tumor was also adherent to the falx and basal dura. He recovered completely following surgery. The postoperative period was uneventful. Histopathology was suggestive of PXA with high mitotic index. There was no recurrence of tumor at 6 months' follow-up. He is under close follow-up and will be offered radiotherapy if the tumor recurs. No PXAs arising from this location have been reported previously, and it is important to be aware of this possibility in children presenting with seizures and MRI which shows a solid cystic lesion superficially located in the ACF base region.