Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis

AIMS: To report the previously undescribed phenomenon of extensive meningeal gliomatosis in a pleomorphic xanthoastrocytoma (PXA) and to examine possible prognostic features. CASE DETAILS: An 11-year-old girl underwent excision of a left temporoparietal pleomorphic xanthoastrocytoma and 8 months later underwent re-excision of a local tumour recurrence. Although mitoses were not present in any of the resection specimens, the development of necrosis and increased immunoreactivity for proliferating cell nuclear antigen (PCNA) indicated anaplastic transformation in the recurrent tumour. Three months later extensive meningeal gliomatosis with minimal local recurrence resulted in death. CONCLUSIONS: Subarachnoid dissemination of a locally recurrent PXA in the absence of mitotic activity is an extremely unusual occurrence. PCNA expression in the initial excision specimens did not predict subsequent anaplastic transformation.