Malignant Transformation of a Rosette-Forming Glioneuronal Tumor with IDH1 Mutation: A Case Report and Literature Review

• Main Authors: Ronie Romelean Jayapalan, Kein Seong Mun, Kum Thong Wong, Sheau Fung Sia
• Format: Article
• Language: English
• Published: Elsevier 2019-04-01
• Series: World Neurosurgery: X
• Online Access: http://www.sciencedirect.com/science/article/pii/S2590139718300061

Background: Rosette-forming glioneuronal tumor (World Health Organization grade I) is considered as a benign tumor with very low potential for progression. The potential for malignant transformation of this tumor is not known and has never been reported before in literature. Case Description: We report a 42-year-old man, diagnosed with rosette-forming glioneuronal tumor of the fourth ventricle with a positive isocitrate dehydrogenase 1 mutation, progressed to glioblastoma after 6 years from diagnosis. We discuss the clinical history, radiological findings, and histopathological characteristic with immunohistochemistry findings observed in this unique case. Conclusions: Despite being acceptable as benign, based on our observations in this case, there is a potential for malignant transformation of rosette-forming glioneuronal tumor. The role of isocitrate dehydrogenase 1 mutation leading to malignant transformation could not be established as our finding is novel and further prospective studies are required to prove this association. Key words: Glioblastoma IDH mutant, IDH 1 mutation, Malignant transformation, RGNT