Atypical papillary glioneuronal tumor

We describe a 34-year-old man who presented with headaches for about 3 months. Magnetic Resonance Imaging (MRI) revealed a large cystic tumor, involving the right frontoparietal lobe region. Pathological study revealed a papillary glioneuronal tumor (PGNT) with mitotic activity and a Ki-67 labeling index of approximately 15%. Five years after radical surgery and radiotherapy, the patient is symptom-free, without tumor recurrence or residual tumor. This case supports the existence of an atypical variant of PGNT, with mitotic activity and high proliferative index, and suggests that in these tumors, histological findings of malignity not necessarily indicate a short-term unfavorable behavior.