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Necrotic rhabdoid meningiomas with aggressive clinical behavior
E Matyja1, W Grajkowska, P Nauman
1Department of Experimental and Clinical Neuropathology, M. Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland. matyja@cmdik.pan.pl
Clinical Neuropathology
|September 24, 2010
Summary
Rhabdoid meningioma (RM) is a rare, aggressive WHO Grade III brain tumor. Extensive necrosis in these primary intracranial tumors predicts a poor prognosis and rapid progression.
Area of Science:
- Neuro-oncology
- Pathology
- Histopathology
Background:
- Rhabdoid meningioma (RM) is a rare, aggressive WHO Grade III malignancy.
- It shares histological features with other rhabdoid tumors and shows a propensity for recurrence and metastasis.
Observation:
- Two unusual cases of primary intracranial rhabdoid meningiomas in adults are presented.
- Tumors were characterized by extensive necrosis and focal clusters of neoplastic cells with rhabdoid morphology.
- Classical meningothelial features were scarce; one case was entirely rhabdoid.
Findings:
- Immunohistochemistry and electron microscopy were crucial for diagnosis.
- Rhabdoid cells showed positivity for vimentin, S-100, EMA, and cytokeratins, with high MIB-1 labeling index (80-90%).
- SNF5 (INI1) protein expression supported the diagnosis; ultrastructural analysis revealed characteristic rhabdoid features.
Implications:
- Extensive necrosis may serve as a predictor of aggressive clinical behavior in rhabdoid meningiomas.
- Accurate diagnosis is critical to differentiate from other neoplasms like AT/RTs and metastatic carcinoma.
- These aggressive tumors were associated with a lethal outcome within months of diagnosis.
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