Rosette-forming glioneuronal tumor (RGNT) is a rare and distinct primary nervous system tumor. The literature on this novel neoplasm is sparse and limited to mostly case reports. Reviews on the characteristics of this tumor are fewer and far between with the latest up to a decade old. We thus provide a comprehensive review of recent literature to characterize presenting symptoms, radiological evidence, treatment options, and prognosis of this novel neoplasm. A Medline search for case reports detailing primary rosette-forming glioneuronal tumors was performed. RGNTs are a benign tumor of indolent course with mixed glial and neurocytic components. There is a slight female predominance with mean age of presentation at 23.57 years. Headaches, visual disturbances, and nausea and vomiting are the most common symptoms. Most RGNTs have solid and cystic components, arising most frequently in the fourth ventricle or cerebellum. Management is usually through surgery with gross total resection (GTR) providing better prognosis.
Keywords: Benign; Brain tumor; Cerebellar mass; Posterior fossa neoplasm; RGNT; Rosette-forming glioneuronal tumor.