PLEOMORPHIC XANTHOASTROCYTOMA REPORT OF 4 CASES, WITH MRI SCAN APPEARANCES AND LITERATURE-REVIEW

Pleomorphic xanthoastrocytoma (PXA) is a rare glial tumour typically o ccurring in young patients in the first three decades, having a superf icial 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 da ys and was aged 2 1/2 years at presentation. This is the youngest pati ent with PXA yet reported. Three of the four patients had seizures, bu t in one case the tumour was not the cause of the seizures. Review of the literature has revealed 47 reported cases. Mean age at presentatio n was 14.3 years. Epilepsy occurred in 78%. Seventeen patients were al ive without recurrence at a mean of 7.9 years after diagnosis and 10 p atients died at a mean of 7.4 years after diagnosis. Thirteen cases ha d recurrence at a mean of 6 years after surgery and in five instances the recurrence was in the form of a glioblastoma. Resections which wer e grossly total were less likely to develop recurrence than those whic h were subtotal. Complete gross resection of tumour offers the best th erapeutic option in PXA.