The authors describe a case of a diffuse primary leptomeningeal oligod
endroglioma in a 17-year-old girl who presented with raised intracrani
al pressure and hydrocephalus. She underwent imaging studies and a lef
t frontotemporal craniotomy that revealed a cystic oligodendroglioma i
n the suprasellar cistern and spread of neoplastic cells to the spinal
leptomeninges. The tumor showed little response to maximum radiothera
py and chemotherapy, and the patient died from complications of high-d
ose chemotherapy 2 years after diagnosis. Postmortem examination of th
e brain and spinal cord revealed diffuse meningeal infiltration by neo
plastic cells and no evidence of an intraparenchymal origin. Glial het
erotopias were noted at several sites along the brain base, adding cir
cumstantial support to the theory that leptomeningeal gliomas are deri
ved from ectopic glial tissue in the subarachnoid space.