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Case report reveals a misidentified meningioma in Erdheim–Chester disease, highlighting histiocytosis implications.
Erdheim–Chester disease (ECD) is a rare non-Langerhans cell systemic histiocytosis affecting multiple organs, more commonly long bones, lungs, heart and less commonly brain. Meningeal involvement is extremely rare, and till now only six cases have been detected. Our patient presented with multiple supra- and infratentorial enhancing meningeal mass lesions disguised as multiple meningiomas. The mass lesion causing the mass effect was excised, and its histopathology showed histiocytosis. Multisystemic evaluation and molecular studies confirmed the diagnosis of ECD, and the patient was started on systemic vemurafenib.
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Mantry et al. (2026) studied this question.
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