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Meningiomas are common central nervous system tumors with a wide range of morphological variants, assigned World Health Organization (WHO) Grades I - III. We report an extremely rare rhabdoid, papillary and clear cell meningioma (WHO Grade III) in a 29-year-old female, who presented with diplopia and headache over a few days, 2 years ago. Magnetic resonance imaging showed a well-circumscribed, lobulated, predominantly solid and contrast-enhancing lesion in the right temporal, parietal and occipital lobes. On routine staining, the tumor did not display classical meningioma features. A wide immunohistochemical panel ruled out metastasis and endorsed the meningothelial nature of the lesion (positivity for epithelial membrane antigen and vimentin). Electron microscopy did not show usual hallmarks of meningioma but was helpful in excluding other tumors. Even though the three variants are associated with aggressive behavior, the patient is currently asymptomatic. The concurrent use of different techniques was essential for diagnosis.

Meningioma with rhabdoid, papillary and clear cell features: case report and review of association of rare meningioma variants