Pleomorphic xanthoastrocytoma (PXA) is a benign glial tumor, the association of which
with neurofibromatosis type I (NF-1) has been often reported in the literature. Although
malignant conversion to glioblastoma may be seen in 5%–10% of PXA, the same has been
reported only once in the presence of NF-1. We report, so far known to be only the
second such case all over. A 25-year-old male, a known case of NF-1, underwent frontal
craniotomy for a superficially located right frontal lesion, histology of which suggested
PXA. Two years later, the lesion recurred and the subsequent surgery revealed malignant
conversion to glioblastoma. After adjuvant radiotherapy, the patient now continues
to do well and is free of disease after another 3 years of follow-up. We believe that
if low levels of neurofibromin are seen in such cases with malignant conversion, subsequently
increased neurofibromin levels may be responsible for better overall survival in these
patients.
Key-words:
Anaplastic - glioblastoma - malignant - neurofibroma - pleomorphic xanthoastrocytoma
