Papillary glioneuronal tumor (PGNT) is a low-grade biphasic tumor that is composed of glial fibrillary acidic protein (GFAP)-positive glial cells and synaptophysin-positive neurons. We report a case of PGNT occurring in the right occipital lobe of a 48-year-old woman who presented with acute headache and left homonymous hemianopsia, the latter of which was difficult to distinguish from malignant brain tumors because of peritumoral brain edema, intratumoral hemorrhage, and intraoperative fluorescence staining. PGNT should be included as one of the differential diagnoses in cases where the tumor shows hemorrhagic change despite decreased perfusion in arterial spin labeling MRI.

The aim of the present study was to determine which individual or combined CpG sites among O⁶-methylguanine DNA methyltransferase CpG 74–89 in glioblastoma mainly affects the response to temozolomide resulting from CpG methylation using statistical analyses focused on the tumor volume ratio (TVR). We retrospectively examined 44 patients who had postoperative volumetrically measurable residual tumor tissue and received adjuvant temozolomide therapy for at least 6 months after initial chemoradiotherapy. TVR was defined as the tumor volume 6 months after the initial chemoradiotherapy divided by that before the start of chemoradiotherapy. Predictive values for TVR as a response to adjuvant therapy were compared among the averaged methylation percentages of individual or combined CpGs using the receiver operating characteristic curve. Our data revealed that combined CpG 78 and 79 showed a high area under the curve (AUC) and a positive likelihood ratio and that combined CpG 76–79 showed the highest AUC among all combinations. AUCs of consecutive CpG combinations tended to be higher for CpG 74–82 in exon 1 than for CpG 83–89 in intron 1. In conclusion, the methylation status at CpG sites in exon 1 was strongly associated with TVR reduction in glioblastoma.