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Suprasellar ganglioglioma with unusual diffuse involvement of the entire optico-chiasmal hypothalamic pathway
Rakesh Jalali1, Chandrashekar E Deopujari, Ritu Bhutani
1Department of Radiation Oncology and Pathology, Tata Memorial Hospital, Mumbai and Neurosurgery, BJ Wadia Hospital, Mumbai, India. rjalali@tmc.gov.in
Insights
This case study highlights an unusual pediatric ganglioglioma (GG) with extensive spread. The rare central nervous system tumor mimicked other conditions but was confirmed by pathology.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Neuroradiology
Background:
- Gangliogliomas (GG) are rare mixed glioneuronal tumors of the central nervous system (CNS).
- They predominantly affect the pediatric population, typically occurring in the temporal lobes.
Observation:
- A 7-year-old child presented with bilateral visual defects and was diagnosed with a sellar/suprasellar mass.
- Brain MRI revealed a diffuse, bilaterally symmetrical lesion involving the optic chiasm, optic nerves, hypothalamus, temporal lobes, thalami, and basal ganglia.
- Radiological features were indistinct from chiasmatic astrocytoma or germ cell tumor.
Findings:
- Histopathological analysis confirmed the diagnosis as ganglioglioma.
- This case presents an unusually widespread diffuse involvement of the entire optico-chiasmal hypothalamic pathway.
- Optic apparatus gangliogliomas are rarely reported, making this presentation unique.
Implications:
- This case expands the known spectrum of ganglioglioma presentation in children.
- It underscores the importance of histopathological confirmation for CNS tumors with atypical radiological features.
- Further research into the behavior and management of extensive gangliogliomas is warranted.
Abstract:
Gangliogliomas (GG) are mixed glioneuronal tumors of the central nervous system (CNS), occurring mostly in the pediatric population, with common sites being temporal lobes and less commonly in the frontal and parietal lobes. We report a case of a 7-year-old child who presented with bilateral visual defects for 6 months. Magnetic resonance imaging (MRI) of the brain revealed an intensely enhancing mass lesion with calcification in the sellar and suprasellar region involving the optic chiasm and the left optic nerve. The mass showed almost bilaterally symmetrical diffuse spread along the optic tracts posteriorly and hypothalamus, temporal lobes, thalami and the basal ganglia. The lesion was radiologically indistinguishable from chiasmatic astrocytoma or a germ cell tumor but histopathological features were of a ganglioglioma. While a few optic apparatus gangliogliomas have been reported in the literature, such widespread diffuse involvement of the entire optico-chiasmal hypothalamic pathway is unusual.
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