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Spinal oligodendroglioma with gliomatosis in a child. Case report
H S Gilmer-Hill1, W G Ellis, S G Imbesi
1Department of Pediatric Neurosurgery, Children's Hospital of Michigan/The Detroit Medical Center, USA.
Insights
This study details a rare pediatric oligodendrogliomatosis case originating from a spinal tumor. Chemotherapy with carboplatin offered long-term palliation and improved quality of life for the child.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Neurosurgery
Background:
- Oligodendrogliomatosis is a rare primary glial tumor, with pediatric cases and spinal origins being exceptionally uncommon.
- This report focuses on a unique case of oligodendrogliomatosis in a child, suspected to have a primary spinal cord tumor.
Observation:
- A 2.5-year-old boy presented with ataxia, neck stiffness, and developmental regression.
- Initial neuroimaging revealed ventriculomegaly and cerebellar cysts, with subsequent MRI showing a cervical intramedullary lesion.
- Follow-up imaging demonstrated diffuse leptomeningeal spread, characterized by small cysts on the brainstem, cerebellum, and cerebral cortices.
Findings:
- Pathological analysis confirmed neoplastic glial cells with characteristic features of oligodendroglioma, migrating within the subpial space.
- The diagnosis of oligodendrogliomatosis cerebri was established, representing a rare occurrence in a pediatric patient with a spinal primary.
- This case is among the few documented instances of childhood oligodendrogliomatosis, particularly with a spinal origin.
Implications:
- This case expands the understanding of oligodendrogliomatosis presentation and behavior in pediatric patients.
- It highlights the potential for spinal cord tumors to disseminate leptomeningeally in children.
- Effective long-term palliation and quality of life were achieved with carboplatin chemotherapy, suggesting its therapeutic role in such rare conditions.
Abstract:
The authors present a rare case of oligodendrogliomatosis in a child, which they believe originated from a primary spinal cord tumor. At 2.5 years of age this boy developed poor balance, neck stiffness, and a regression in developmental milestones. A computerized tomography (CT) scan of the head initially revealed ventriculomegaly and multiple cystic cerebellar lesions. In addition, magnetic resonance (MR) imaging revealed a cystic intramedullary lesion involving the cervical spinal cord. A CT scan of the head and an MR image obtained 3 years later demonstrated diffuse small cysts on the surface of the brainstem, cerebellum, medial temporal and inferior frontal cortices, subcortical white matter, and corpus callosum suggestive of leptomeningeal tumor spread. Analysis of pathological specimens obtained at surgery showed neoplastic glial cells with small, uniform nuclei and perinuclear clear zones. The cells appeared to migrate along the subpial space but no tumor cells were present in the subarachnoid space. These findings were compatible with a diagnosis of oligodendrogliomatosis cerebri. Despite having a complicated course, chemotherapy with carboplatin has provided the patient with long-term palliation and a high quality of life. This case may represent the fifth report in the literature of oligodendrogliomatosis occurring in a child but only the third occurring with a spinal primary tumor.
