Primary diffuse multinodular leptomeningeal gliomatosis: case report and review of the literature.

BACKGROUND

Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have been reported in the literature. We report a recent case and compare data with previously published observations.

METHODS

A 50-year-old man was admitted to the neurosurgery department for a previous 4-month history of headache, associated with nonspecific neurological signs. Biologic data and cerebrospinal fluid examination suggested an inflammatory process. The patient was given an antituberculous therapy. Magnetic resonance imaging revealed a multinodular enhancement of spinal nerve roots. A biopsy of sacral rootlets was performed. Histological examination revealed an anaplastic astrocytoma. Patient's status worsened, and death occurred 7 months later.

RESULTS

Complete neuraxis postmortem examination revealed no intraparenchymatous glioma and was conclusive for the diagnosis of primary leptomeningeal gliomatosis (astrocytic, World Health Organization grade III), with a multinodular pattern in the spinal cord, the brainstem, and the brain base with diffuse extension into the cerebellar subarachnoid spaces.

CONCLUSIONS

Our case illustrates the diagnostic difficulties in making the premortem diagnosis. The review of the literature indicates that there are no specific clinical or biologic signs. Magnetic resonance imaging using T1-weighted images with gadolinium enhancement and biopsy material may be useful diagnostic tools. In most cases, autopsy evaluation alone permits definitive primary diffuse leptomeningeal gliomatosis diagnosis. Whatever the histological characteristics of proliferating cells are, the prognosis remains poor. No prognostic factors have been shown to be correlated with survival time. Unfortunately, no routine treatment has been yet proposed.