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 de
velopmental milestones. A computerized tomography (CT) scan of the head ini
tially 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 surfa
ce of the brainstem, cerebellum medial temporal and inferior frontal cortic
es, subcortical white matter, and corpus callosum suggestive of leptomening
eal tumor spread. Analysis of pathological specimens obtained at surgery sh
owed neoplastic glial cells with small, uniform nuclei and perinuclear clea
r zones. The cells appeared to migrate along the subpial space but no tumor
cells were present in the subarachnoid space. These findings were compatib
le with a diagnosis of oligodendrogliomatosis cerebri. Despite having a com
plicated course, chemotherapy with carboplatin has provided the patient wit
h 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.