Erdheim-Chester disease of the brain: cytological features and differential diagnosis of a challenging case.
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.
Rushing
Elisabeth J
EJ
Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA. rushinge@afip.osd.mil
Kaplan
Keith J
KJ
Mena
Hernando
H
Sandberg
Glenn D
GD
Koeller
Kelly
K
Bouffard
John-Paul
JP
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