Rhabdoid Meningiomas: Histopathological Diagnosis
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Rhabdoid meningioma (RM) is an uncommon, aggressive variant of meningioma, designated as WHO grade III malignancy. It is characterized by a high risk to local recurrences, neoplastic dissemination and remote metastases. So far, the majority of reported cases of RMs have been found as secondary rhabdoid lesions in tumour recurrences.
The histological pattern of RM is characterized by the presence of neoplastic cells of distinctive rhabdoid morphology associated with polyphenotypic immunohistochemical profile. Rhabdoid cells usually stain diffusely for vimentin and S-100 protein and show focal expression of epithelial membrane antigen and cytokeratins. The tumour exhibits brisk mitotic activity and high MIB-1 labelling index.
The accurate histopathological diagnosis of rhabdoid subtype of meningioma is often difficult because of its similarity to other primary or secondary brain tumours that exhibit rhabdoid morphology. Particularly, the distinction from atypical teratoid/rhabdoid tumours (AT/RTs) and metastatic carcinomas is challenging and requires a wide spectrum of immunohistochemical and electron microscopic studies. The diagnosis of rhabdoid meningioma might be supported by evidence of SNF5 (INI1) protein expression, that allows to exclude AT/RT. Ultrastructural features characteristic for meningothelial cell phenotype i.e. interdigitating cell processes, numerous desmosomes and paranuclear whorls of intermediate filaments might be helpful in confirming the diagnosis.
KeywordsRhabdoid Tumour Choroid Plexus Carcinoma Rhabdoid Cell Rhabdoid Feature Meningothelial Cell
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