Erdheim-Chester Disease (ECD) is a rare non-Langerhans' cell histiocytosis described in 1930 by Jakob Erdheim and William Chester, it can present as a multisystemic entity that forms xanthogranulomas which are foamy histiocytes surrounded by fibrotic tissue. Lesions are commonly located in long bones, Central Nervous System (CNS), cardiovascular system, lungs, kidneys and skin. The CNS is involved in approximately 50% of cases and can compromise both extra and intra-axial structures and therefore can mimic schwannomas or meningiomas, amongst other mass lesions. Clinical presentation will differ from patient to patient thus diagnosis depends greatly in imaging, immunohistochemistry and genetic findings within the pathology analysis. The pathogenesis of this disease remains unknown. It is most commonly found in the middle-aged male population. Here, we present a case of a middle-aged woman with an extra-axial lesion that was initially considered to be neurosarcoidosis proving the diagnostic challenge this entity implies.
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