Background
Papillary glioneuronal tumors (PGNT) are rare, with only around 140 cases being reported since this tumor was first described in the fourth edition of the WHO classification of tumors of the central nervous system.
Case Presentation
We report the case of a 41-year-old male who presented with a right frontal tumor. The only presenting complaint was seizures. A cystic tumor with an enhancing nodule was found on MRI scan and this was excised via a craniotomy. Intra-operatively the cyst contents were thick and mucinous, the nodule was vascular and the cyst walls were made up of compressed white matter.
Conclusion
PGNT is a low grade (WHO grade I) tumor that usually affects younger patients. They are usually periventriuclar in location and the presence of thick cyst contents may warn that the lesion is not a hemangioblastoma or dysembryoplastic neuroepithelial tumor (DNET). When in doubt, a frozen section may help to decide if the cyst wall should be excised. These tumors have a good prognosis if total excision is achieved.