Pleomorphic xanthoastrocytoma--report of four cases, with MRI scan appearances and literature review

Abstract

Pleomorphic xanthoastrocytoma (PXA) is a rare glial tumour typically occurring in young patients in the first three decades, having a superficial cortical location and with a relatively good prognosis for long-term survival. Four cases are reviewed. The magnetic resonance imaging (MRI) appearances, which in PXA have been reported only once before, are described in three cases. The fourth case was studied by computed tomography and angiography. One patient developed seizures at age 2 days and was aged 2 1/2 years at presentation. This is the youngest patient with PXA yet reported. Three of the four patients had seizures, but in one case the tumour was not the cause of the seizures. Review of the literature has revealed 47 reported cases. Mean age at presentation was 14.3 years. Epilepsy occurred in 78%. Seventeen patients were alive without recurrence at a mean of 7.9 years after diagnosis and 10 patients died at a mean of 7.4 years after diagnosis. Thirteen cases had recurrence at a mean of 6 years after surgery and in five instances the recurrence was in the form of a glioblastoma. Resections which were grossly total were less likely to develop recurrence than those which were subtotal. Complete gross resection of tumour offers the best therapeutic option in PXA.