Giant cell glioblastoma with unique bilateral cerebellopontine angle localization considered as extraaxial tumor growth in a patient with neurofibromatosis Type 1

Abstract

Giant cell glioblastoma multiforme (GCGBM) is a rare variant of glioblastoma, occurring predominantly in the cerebral hemispheres. Its infratentorial localization has been documented occasionally, while GCGBM in the cerebellopontine angle (CPA) region has not been described so far. We report a case of GCGBM presenting primarily as an extraaxial bilateral CPA tumor in a 29-year-old woman with neurofibromatosis Type 1 (NF1). The patient died shortly after surgery of the right CPA tumor. Postmortem study of the brain revealed large tumor masses, located in the CPA bilaterally, encasing the brainstem base and cisternal portions of the cranial nerves. Tumor masses were demarcated from the brainstem and cerebellum and covered by leptomeninges. Microscopically, a slight subpial tumor seeding from the leptomeninges into the brain parenchyma was observed in the right CPA region. The tumor showed highly pleomorphic, giant and multinucleated cells, densely cellular sheets of poorly differentiated cells and pseudopalisading necroses. Tumor cells were positive for GFAP, S-100 protein, and p53 and negative for neuronal antigens. The MIB-1 labeling index was very high in densely cellular areas. To our knowledge this is the second report of GCGBM in an NF1 patient and the first reported case of GCGBM presenting as an extraaxial leptomeningeal lesion with bilateral CPA localization, which might be considered as primary leptomeningeal gliomatosis.

An integrative survival analysis and a systematic review of the cerebellopontine angle glioblastomas

“…Molecular profiling of the CPA glioblastoma has revealed underlying genetic mutations in the TP53, TERT, NF1, and RB1 genes 11 . Taraszewska et al 12 reported bilateral CPA glioblastoma in a patient with neurofibromatosis type 1, suggesting a possible genetic link. The mean age in our cohort was lower when compared with the reported age in the literature, with a slight male predominance, usually observed in glioblastomas 1,7 .…”

Giant cell glioblastoma in a child with clinical and family history of neurofibromatosis

“…The first case was reported by Kroh et al in 2004 in an 8-year-old child, [3] and the second case was reported by Taraszewska et al in a 29-year-old female in 2013. [4] Histopathologically, GCGs correspond to WHO Grade IV.…”

“…To the best of our knowledge, this is the third case report of GCG associated with clinical features of NF, and the second case report in a child in a scenario of NF type 1 (NF1). [3,4] In this case report, we discuss GCG with a review of the relevant literature.…”

Ten Years of Disease-free Survival of a Giant Cell Glioblastoma Considered Initially as a Convexity Meningioma

“…2,3 Such a radiologic finding is exceedingly rare in giant cell glioblastoma, with only 2 cases recently described in the English medical literature. 4,5 Such a diagnosis, erroneously suspected, could have entailed in inadequate therapy; that is why we want to stress the importance of combined application of multiple MRI techniques including routine sequences and MR spectroscopy, diffusion and perfusion as a preferred policy in the exploration of brain tumors and assessment of differential diagnosis.…”