Papillary Glioneuronal Tumor

Objective and importance Papillary glioneuronal tumor is a recently described neoplasm composed of gliovascular pseudopapillae associated with intervening neuronal cells ranging from neurocytes to ganglion cells. This tumor is not currently included in the WHO classification of tumors of the central nervous system. We describe a new case of papillary glioneuronal tumor and analyze the data for a series of further 15 patients from international literature.Clinical presentation A 27-year-old man presented to us for generalized seizure. CT and MRI showed a cystic tumor with mural nodule in the left frontal lobe.Intervention Frontal craniotomy with gross total removal of the tumor was performed. Histopathological examination was positive for papillary glioneuronal tumor.Conclusion The clinical, radiologic, and pathological features of our case are strikingly similar to those of the previous reported cases. A review of the literature disclosed only 15 other cases of these tumors.It is important that every new case of PGNT is reported to allow its recognition and classification.