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Papillary glioneuronal tumor (PGNT) is a rare cerebral tumor that is most commonly found in the frontal lobe of young adults. It has been designated as a World Health Organization (WHO) grade I lesion because it usually shows low Ki-67 labeling indices of 1% to 2% and generally does not recur after resection (1). Here we report follow-up data on a patient with an unusual atypical PGNT with anaplastic histologic features. The case was first diagnosed by the late Dr. Allan J. Yates and was the subject of a case report in 2008 (2).A 19-year-old woman presented with a 5.0-cm left parietotemporal contrast-enhancing lesion (Fig. 1A). The gross total resection specimen showed typical PGNT histology, ie, glial fibrillary acidic protein (GFAP)-immunoreactive papillary regions (Fig. 1B–D) and synaptophysin-positive solid regions containing scattered NeuN-immunoreactive ganglion-like cells (Fig. 1E–G). Neurofilament immunostain was negative. The tumor also demonstrated a high Ki-67 labeling index of 26%, mitotic activity, focal vascular proliferation (2), fibrin thrombi and necrosis (Fig. 1D). Postoperatively, the patient was treated with conformational radiation and low-dose temozolomide (TMZ) for 4 weeks; this was followed by standard-dose TMZ for 13 months (2).FIGURE 1 Imaging and histology of the intracranial tumor (A–G) , intracranial recurrence (H) , …

Anaplastic Papillary Glioneuronal Tumor With Extraneural Metastases