Primary Leptomeningeal Gliomatosis: Case Report

The patient, a 15-year-old female, was admitted because of occipitalgia of one year's duration. CT scan revealed contrast enhancement resembling tuberculous meningitis or pineal region tumor in the cisterns of the skull base as well as in the pineal region. Cytological examination of the CSF showed clusters of atypical cells and brain tumor was suspected. Intrathecal injection of ACNU and radiotherapy showed some effect on neurological and CT scan findings, but the enhancement in the cisterns again gradually increased in size. The clinical course was downhill and the patient expired 2 years after admission. Autopsy revealed diffuse proliferation of atypical astrocytic cells consistent with astrocytoma Grade II in the leptomeninges of entire central nervous system. No gliomatous changes were noted in the brain and spinal parenchyma, and the diagnosis of primary diffuse leptomeningeal gliomatosis was made. Primary leptomeningeal gliomatosis is rare and only 16 cases are reported in the literature. The diffuse form especially rare and this was the fourth case, so far reported.