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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:134-137, January 2004
© 2004 American Society of Neuroradiology

Case Report
BRAIN

Erdheim-Chester Disease Mimicking Multiple Meningiomas Syndrome

Mahlon D. Johnson^a, Joseph P. Aulino^b, Madan Jagasia^c and Louise A. Mawn^d

^a Department of Pathology, Vanderbilt Medical School, Nashville, TN
^b Department of Radiology and Radiological Sciences, Vanderbilt Medical School, Nashville, TN
^c Department of Hematology and Oncology, Vanderbilt Medical School, Nashville, TN
^d Department of Ophthalmology, Vanderbilt Medical School, Nashville, TN

Address correspondence to Dr. Mahlon D. Johnson, Department of Pathology, Vanderbilt Medical School, 21st Ave. S, Nashville, TN 37232

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.