Intracranial Erdheim-Chester Disease

ABSTRACT

Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis consisting of disseminated xanthogranulomatous infiltration and fibrosis that involves the long bones, visceral organs, orbital, retroperitoneal and soft tissues. Intracranial involvement is very rare, although the most common site is extra axial if it occurs. In our case study, a 53-year-old woman with one-month history of left hemianopsia was examined and treated. Her initial T2-weighted MRI revealed increase in signal intensity and an irregularly enhanced mass following gadolinium injection in the right temporal lobe. Stereotactic biopsy was done, and histopathological diagnosis was Erdheim- Chester disease, consist of sheets of foamy histiocytes with abundant cytoplasm. The patient was treated with oral corticosteroid for 4 weeks. During next 6 months, the clinical picture and the MRI showed improvements.