Spinal cord ganglioglioma in a child with neurofibromatosis Type 2

Case report and literature review

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✓ Gangliogliomas of the spinal cord are rare disease entities that occur in early childhood. Their occurrence in association with neurofibromatosis Type 2 (NF2) has not been described. The authors describe the unique case of a 2-year-old child with stigmata of NF2 who harbored a spinal cord ganglioglioma that presented as a rapidly growing, exophytic intramedullary mass lesion at the cervicomedullary junction. Treatment consisted of complete surgical resection. Histopathological analysis of the lesion demonstrated a mixed population of neoplastic cells, of both neuronal and glial lineage, that supported the diagnosis of ganglioglioma.

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Current address for Dr. Sawin: Florida Neurosurgery, PA, Winter Park, Florida.

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    Magnetic resonance images of the cervical spine and brainstem obtained in the child at age 25 months immediately before surgical intervention. Sagittal T1-(left) and T2-(center) weighted MR images revealing an isointense mass dorsolateral to the cervical spinal cord that extends from the cervicomedullary junction to the C-4 level. Minimal peripheral enhancement of the tumor is observed after intravenous administration of gadolinium (right).

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    Photomicrograph of the tumor specimen. Note the biphasic nature of the cellular population, with large atypical ganglion cells and smaller neoplastic astrocytes with hyperchromatic nuclei. H & E, original magnification × 250.

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