J Korean Neurosurg Soc.  2004 Dec;36(6):496-498.

Intracranial Erdheim-Chester Disease

Affiliations
  • 1Department of Neurosurgery, College of Medicine, In-je University, Ilsan Paik Hospital, Goyang, Korea. nhyoo00@hanmail.net

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.

Keyword

Erdheim-Chester disease; Intracranial; Histiocytosis

MeSH Terms

Biopsy
Cytoplasm
Diagnosis
Erdheim-Chester Disease*
Female
Fibrosis
Gadolinium
Hemianopsia
Histiocytes
Histiocytosis
Humans
Magnetic Resonance Imaging
Middle Aged
Orbit
Temporal Lobe
Gadolinium
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