Clinicopathological features of gliomatosis cerebri: a case report and review of literatures

Abstract

Objective To explore the clinicopathological features of gliomatosis cerebri. Methods The clinical manifestations, neuroimaging, histopathological and immunohistochemical features were analysed in one case of gliomatosis cerebri. Related literatures were reviewed. Results A 24⁃year⁃ old man presented with a seizure which started as paroxysmal amaurosis and progressed to loss of consciousness and severe headache. The magnetic resonance imaging (MRI) showed a diffuse slight hyperintensity in the left temporoparietal and basal ganglial region. The magnetic resonance spectroscopy (MRS) showed an increased Cho/NAA ratio. The patient subsequently underwent a craniotomy with part of left temporal tissue and hippocampus resection. Microscopically, there was diffuse infiltration of the brain parenchyma by low to moderate cellularity of astrocyte ⁃ like cells with elongated, fusiform and mildly hyperchromatic nuclei. Mitotic figure was rare. Microvascular proliferation and necrosis were absent. Secondary structures, including subpial and subependymal condensation, perivascular aggregates and perineuronal satellitosis were evident. The distinction between grey and white matter was blurred. There was very little destruction of the pre ⁃ existing parenchyma. On immunohistochemical examination, the neoplasm was reactive for glial fibrillary acidic protein (GFAP), S ⁃ 100 protein (S ⁃ 100) and negtive for oligodendrocyte lineage transcription factor 2 (Olig ⁃ 2), synaptophysin (Syn) and neuronal nuclei (NeuN). TP53 protein was overexpressed in 8% of tumor cells. Ki ⁃ 67 antigen labeled index was about 10% . Conclusion Gliomatosis cerebri is an unusual and aggressive glial neoplasm with infiltrative involvement of at least three cerebral lobes. There is minimal mass effect by neuroimaging, but MRI and MRS findings can suggest the diagnosis of gliomatosis cerebri. Histologically, the tumor cells are diffuse infiltrative and may form secondary structures. The differential diagnosis include multicentric/multifocal glioma and demyelinating disease and so on. TP53 immunoreactivity and increased Ki⁃67 antigen labeled index are important for distinguishing gliomatosis cerebri from other non⁃neoplastic diseases.

DOI：10.3969/j.issn.1672-6731.2011.02.023