Rosette-Forming Glioneuronal Tumor at Septum Pellucidum: Insights Gained from a Common Tumor at Rare Location

Abstract

The rosette-forming glioneuronal tumor (RGNT) is an uncommon entity and carries a special character because of its mixed glial and neuronal composition in the histomorphological appearance. These lesions have a benign character and carry a good outcome if undergoes gross total resection. Over the past 15 years, there have been a significant change in their nomenclature depending upon the location to histological composition. Herein, we report an interesting case of a 26-year-old lady who was diagnosed to have the lesion at the septum pellucidum with significant symptoms in the form of headache and seizure episodes. A gross total resection was achieved and she made an uneventful recovery. We discuss the literature on the incidence, location, and histological characteristics of the RGNT in various age groups.

Keywords

glioneuronal tumor - septum pellucidum - rosette-forming

• References

• 1 Nagaishi M, Nobusawa S, Matsumura N. et al. SLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors. J Clin Neurosci 2016; 23 (01) 73-75
• 2 Louis DN, Ohgaki H, Wiestler OD. et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 2007; 114 (02) 97-109
• 3 Louis DN, Perry A, Reifenberger G. et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 2016; 131 (06) 803-820
• 4 Xiong J, Liu Y, Chu SG. et al. Rosette-forming glioneuronal tumor of the septum pellucidum with extension to the supratentorial ventricles: rare case with genetic analysis. Neuropathology 2012; 32 (03) 301-305
• 5 Sharma P, Swain M, Padua MD, Ranjan A, Lath R. Rosette-forming glioneuronal tumors: a report of two cases. Neurol India 2011; 59 (02) 276-280
• 6 Komori T, Scheithauer BW, Hirose T. A rosette-forming glioneuronal tumor of the fourth ventricle: infratentorial form of dysembryoplastic neuroepithelial tumor?. Am J Surg Pathol 2002; 26 (05) 582-591
• 7 Yang C, Fang J, Li G. et al. Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. Oncotarget 2017; 8 (65) 109175-109190
• 8 Jayapalan RR, Mun KS, Wong KT, Sia SF. Malignant transformation of a rosette-forming glioneuronal tumor with IDH1 mutation: a case report and literature review. World Neurosurg X 2019; 2: 100006
• 9 Al Krinawe Y, Esmaeilzadeh M, Hartmann C, Krauss JK, Hermann EJ. Pediatric rosette-forming glioneuronal tumor of the septum pellucidum. Childs Nerv Syst 2020; 36 (11) 2867-2870
• 10 Jacques TS, Eldridge C, Patel A. et al. Mixed glioneu- ronal tumour of the 4th ventricle with prominent rosette formation. Neuropathol Appl Neurobiol 2006; 32: 217-220
• 11 Scheithauer BW, Silva AI, Ketterling RP, Pula JH, Lininger JF, Krinock MJ. Rosette forming glioneu- ronal tumor: report of a chiasmal-optic nerve example in neurofibromatosis type 1: special pathology report. Neurosurgery 2009; 64: E771-E772
• 12 Anan M, Inoue R, Ishii K. et al. A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosetteforming glioneuronal tumor 217 originat- ing from the spinal cord. Hum Pathol 2009; 40: 898-901
• 13 Frydenberg E, Laherty R, Rodriguez M, Ow-Yang M, Steel T. A rosette-forming glioneuronal tumour of the pineal gland. J ClinNeurosci 2010; 17: 1326-1328
• 14 Solis OE, Mehta RI, Lai A. et al. Rosette-forming glio-neuronal tumor: a pineal region case with IDH1 and IDH2 mutation analyses and literature review of 43 cases. J Neurooncol 2010; 102: 477-484
• 15 Sekar A, Rudrappa S, Gopal S, Ghosal N, Rai A. Rosette-Forming Glioneuronal Tumor in Opticochiasmatic Region—Novel Entity in New Location. World Neurosurg 2019; 125: 253-256