Rosette forming glioneuronal tumor in the optico-chiasmatic region-a novel entity in a new location.

Related Articles

Rosette forming glioneuronal tumor in the optico-chiasmatic region-a novel entity in a new location.

World Neurosurg. 2019 Feb 18;:

Authors: Sekar A, Rudrappa S, Gopal S, Ghosal N, Rai A

Abstract
OBJECTIVE: Rosette forming glioneuronal tumour [RGNT] is a relatively rare entity first identified as a separate entity in 2002 by Komori et al[1]. We are reporting the second case of RGNT in optico-chiasmatic region.
METHOD: A case report with literature review of RGNT with syndromic association.
RESULTS: 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. Till date only one case of RGNT involving the optico-chiasmatic region has been reported in a patient with Neurofibromatosis Type 1. Genetic analysis of this rare tumour identified three 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.
CONCLUSION: To the best of our knowledge, we report the first case of RGNT involving the optico-chiasmatic region without any syndromic association. Other cases of RGNT with syndromic associations provide us with an insight of possible therapeutic interventions.

PMID: 30790744 [PubMed - as supplied by publisher]

from A via a.sfakia on Inoreader https://ift.tt/2tylOYC