title: Erdheim-Chester disease of the brain: cytological features and differential diagnosis of a challenging case.
abstract: Erdheim-Chester disease (ECD) is an uncommon, systemic xanthogranulomatous disorder, with distinct clinicopathological features, that is rarely expected preoperatively. We describe a case that presented in the brain of a 26-yr-old male patient and clinically mimicked the appearance of a neoplasm. The final diagnosis was a surprise. In retrospect, the diagnosis was suggested by the intraoperative "squash" preparations, which demonstrated a mixed cellular proliferation of lymphohistiocytic elements and large, multinucleated cells with vesicular nuclei, prominent nucleoli, and abundant cytoplasm. To the best of our knowledge, this is the first report detailing the cytopathological features of ECD.
authors.affiliation: Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA. rushinge@afip.osd.mil
authors.forename: Elisabeth J
authors.initials: EJ
authors.lastname: Rushing
authors.forename: Keith J
authors.initials: KJ
authors.lastname: Kaplan
authors.forename: Hernando
authors.initials: H
authors.lastname: Mena
authors.forename: Glenn D
authors.initials: GD
authors.lastname: Sandberg
authors.forename: Kelly
authors.initials: K
authors.lastname: Koeller
authors.forename: John-Paul
authors.initials: JP
authors.lastname: Bouffard
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D003581:Cytodiagnosis; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008297:Male; D011859:Radiography