Bilateral Optic Pathway Ganglioglioma: The Fifth Case in the Literature

Abstract

Gangliogliomas are mixed tumors which contain both glial and neuronal elements. The optic pathway is a very rare location for gangliogliomas, with less than 23 cases reported in the literature. Bilateral involvement of the entire optic pathway was reported in only 4 cases before. Because of similar radiological appearance of other pathological entities such as gliomas and craniopharyngiomas, histopathological diagnosis is essential. We report a ganglioglioma case that involved both optic pathways. A 12-year-old patient suffering from visual deterioration for 6 months was evaluated. After a visual field test and radiological examinations, a microsurgical biopsy procedure was performed. Pathological examination revealed dysplastic/neoplastic ganglion cells and neoplastic glial cells, and the diagnosis was a World Health Organization (WHO) grade 1 ganglioglioma. The patient is scheduled for adjuvant radiotherapy with the hope of prevention of progression.

Comparison of two surgical methods for the treatment of optic pathway gliomas in the intraorbital segment: an analysis of long-term clinical follow-up, which evaluates the surgical outcomes

“…The onset of these tumors is usually indicated by unilateral ocular symptoms, and exophthalmos and decreased vision are common clinically. It is relatively rare for the tumor to be in both eyes (9). There are still controversies about the timing and the effects of surgery.…”

BRAF V600E-Mutated Ganglioglioma of the Optic Pathway: A Case Report and Review of the Literature

“…In rare cases, gangliogliomas can also present with bilateral vision changes, including visual field defects or cranial nerve palsies as a result of increased intracranial pressure. The most commonly involved location is the optic nerve and the optic chiasm, although the entire optic pathway can be involved (9,27,28). In cases of optic pathway gangliogliomas, a careful neuro-ophthalmological examination is warranted.…”

“…These tumors can be found anywhere throughout the CNS but over 70% are localized to the temporal lobe (6). Optic nerve gangliogliomas reported in the literature thus far have been low-grade (9–24,26–29) with the exception of 1 report of a high grade teratoid/rhabdoid tumor arising from a recurrent extensive optic pathway ganglioglioma (25). The neuronal component is comprised of haphazardly oriented large dysplastic neurons with perimembranous aggregation of Nissle substance and occasional 2 nuclei with prominent nucleoli.…”