Infratentorial pleomorphic xanthoastrocytoma: report of two cases and review of literature

Pleomorphic xanthoastrocytoma (PXA) typically has benign histology, but may show different prognosis and clinical characteristics in atypical locations. A case each of cerebellar PXA and IV ventricle PXA is reported along with a review of the rare presentations and atypical features of this tumor. A review of the available literature dating back to 1999 revealed 25 cases of PXA in the posterior fossa, of which 17 were in adults, with an average age of 31 years, while supratentorial forms had a younger age profile (26.2 years). PXA in the posterior fossa had a higher rate of recurrence, mortality and malignant progression. In contrast, patients with PXA-ganglioglioma and PXA-pilocytic astrocytoma had good prognosis. The clinicopathological features of infratentorial PXA differ from PXA located in the cerebral hemispheres. Recognizing these atypical manifestations in the different subgroups of PXA is critical for accurate diagnosis and treatment.