Benign and malignant pathology in neurofibromatosis type 1.

A 25-year-old man presented for evaluation of aphasia and generalized seizures. Examination revealed café au lait macules, axillary freckling, and bilateral Lisch nodules. Surgical history included excision of an upper extremity plexiform neurofibroma and optic glioma resection followed by whole-brain radiation 20 years previously. His mother and maternal grandmother had also been diagnosed with neurofibromatosis type 1 (NF1). Recent onset signs and symptoms were attributed to the frontal mass, biopsy proven to be gliosarcoma. Malignant gliomas can arise as a late consequence of whole-brain radiotherapy or result from malignant degeneration of previously benign tumors in patients with NF11,2 figure.