Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression.

OBJECTIVE AND IMPORTANCE: Rhabdoid meningioma (RM) is a relatively new, Grade III tumor entity according to the latest WHO classification. We report rhabdoid and partly papillary, highly anaplastic, intracerebral meningioma with diffuse leptomeningeal spread and distant SCF metastasis to the cervical cord. CLINICAL PRESENTATION: This 27-year-old female was admitted to the hospital with radiological findings suggestive of a primary brain tumor or a metastasis. After subtotal resection and during radiotherapy, follow-up MRI revealed recurrence, metastasis to meninges at the high cervical level, and diffuse basal leptomeningeal enhancement indicating infiltrating tumor. She died approximately 3 months after onset of symptoms. RESULTS: Histological examination revealed rhabdoid and papillary meningioma with high proliferation rate (80% of MIB1-positive cells), necrosis and extensive brain invasion. It was positive for vimentin and S-100 protein, showed focal epithelial membrane antigen expression and accumulation of intermediate filaments on ultrastructural examination. The recurrent tumor diffusely infiltrated leptomeninges and subarachnoid space. CONCLUSION: This is a rare example of mixed, rhabdoid and papillary variant of meningioma, located entirely within the brain parenchyma and accompanied by a fulminant clinical course. The combination of the histological anaplasia with the highest reported proliferation rate, and loss of the cohesion of neoplastic cells led to diffused infiltration of the leptomeninges and metastasis to the spinal cord.