Anaplastic Pleomorphic Xanthoastrocytoma in a Case of Neurofibromatosis Type 1: A Case Report

Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour comprising only <1% of primary brain tumours which is seen in children and young adults. Only 9-20% of the PXA shows anaplastic features and this has a bad prognosis. PXA is a WHO grade II tumour while anaplastic PXA is a WHO grade III tumour. Neurofibromatosis type 1(NF1), which is an autosomal dominant condition, predisposes to tumours of the central nervous system; most of which are pilocytic astrocytomas. Association of PXA with NF1 is very rare and only a very few cases have been reported. Here, we present a case of 42-year-old male, a known case of NF1, with multiple neurofibromas, who presented with right sided hemiparesis, seizures and vomiting. The histopathology and immunohistochemistry features were suggestive of anaplastic PXA.