Erdheim-Chester Disease Mimicking Multiple Meningiomas Syndrome

Abstract

Summary: We describe a rare case of non-Langerhans histiocytosis, consistent with Erdheim-Chester disease (ECD), which presented with lesions resembling multiple meningiomas. The patient was initially evaluated for migraine headaches. Initial MR imaging demonstrated a parasellar mass and a second mass near the torcula considered to represent meningiomas. Within 1 year, he developed bilateral orbital lesions surrounding both optic nerves, which were also considered meningiomas. Biopsy of one orbital mass revealed a non-Langerhans histiocytosis. Subsequently, soft tissue masses, a pericardial effusion, and bone lesions consistent with ECD were identified.