Spinal oligodendroglioma with gliomatosis in a child

Case report

Holly S. Gilmer-Hill Department of Pediatric Neurosurgery, Children's Hospital of Michigan/The Detroit Medical Center, Detroit, Michigan; Departments of Neurology, Pathology, and Neurological Surgery, University of California, Davis Medical Center, Sacramento, California; and Department of Neuroradiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania

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William G. Ellis Department of Pediatric Neurosurgery, Children's Hospital of Michigan/The Detroit Medical Center, Detroit, Michigan; Departments of Neurology, Pathology, and Neurological Surgery, University of California, Davis Medical Center, Sacramento, California; and Department of Neuroradiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania

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Steven G. Imbesi Department of Pediatric Neurosurgery, Children's Hospital of Michigan/The Detroit Medical Center, Detroit, Michigan; Departments of Neurology, Pathology, and Neurological Surgery, University of California, Davis Medical Center, Sacramento, California; and Department of Neuroradiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania

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James E. Boggan Department of Pediatric Neurosurgery, Children's Hospital of Michigan/The Detroit Medical Center, Detroit, Michigan; Departments of Neurology, Pathology, and Neurological Surgery, University of California, Davis Medical Center, Sacramento, California; and Department of Neuroradiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania

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U 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.

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