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Rosette-Forming Glioneuronal Tumor in Opticochiasmatic Region-Novel Entity in New Location.
- Source :
-
World neurosurgery [World Neurosurg] 2019 May; Vol. 125, pp. 253-256. Date of Electronic Publication: 2019 Feb 18. - Publication Year :
- 2019
-
Abstract
- Background: Rosette-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.<br />Case Description: We report a case report and literature review of RGNT with syndromic association.<br />Conclusions: Although 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.<br /> (Copyright © 2019 Elsevier Inc. All rights reserved.)
- Subjects :
- Adolescent
Cerebral Ventricle Neoplasms surgery
Follow-Up Studies
Fourth Ventricle surgery
Ganglioglioma surgery
Humans
Male
Neuroglia pathology
Neurons pathology
Optic Chiasm surgery
Cerebral Ventricle Neoplasms diagnostic imaging
Fourth Ventricle diagnostic imaging
Ganglioglioma diagnostic imaging
Optic Chiasm diagnostic imaging
Rosette Formation
Subjects
Details
- Language :
- English
- ISSN :
- 1878-8769
- Volume :
- 125
- Database :
- MEDLINE
- Journal :
- World neurosurgery
- Publication Type :
- Academic Journal
- Accession number :
- 30790744
- Full Text :
- https://doi.org/10.1016/j.wneu.2019.02.003