• Arunkumar Sekar, Satish Rudrappa, Swaroop Gopal, Nandita Ghosal, and Abhishek Rai.
• Department of Neurosciences, Sakra World Hospital, Bellandur, Bangalore, Karnataka, India.
• World Neurosurg. 2019 May 1; 125: 253-256.

BackgroundRosette-forming glioneuronal tumour [RGNT] is a relatively rare entity first identified as a separate entity in 2002. We are reporting the second case of RGNT in the opticochiasmatic region.Case DescriptionWe report a case report and literature review of RGNT with syndromic association.ConclusionsAlthough initial reports were predominantly in the fourth ventricle, many recent reports have identified the possibility of its occurrence outside fourth ventricle in pineal gland, spinal cord, septum pellucidum, lateral ventricle, and suprasellar region. To date, only 1 case of RGNT involving the opticochiasmatic region has been reported in a patient with neurofibromatosis type 1. Genetic analysis of this rare tumor identified 3 hotspots involving somatic mutations of FGFR-1 and PIK3CA and a germline mutation involving PTPN11, which can be targets for therapeutic intervention in cases where complete resection is not possible. To the best of our knowledge, we report the first case of RGNT involving the opticochiasmatic region without any syndromic association. Other cases of RGNT with syndromic associations provide us with insight into possible therapeutic interventions.Copyright © 2019 Elsevier Inc. All rights reserved.

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