title: Erdheim-Chester disease: imaging-guided therapeutic approach.
abstract: Erdheim-Chester disease (ECD) is a rare form of systemic non-Langerhans cell histiocytosis with characteristic bone involvement. However, extraskeletal involvement occurs in approximately half of the patients. Because of its protean findings, the diagnosis of ECD is often delayed; thus, a clinical suspicion may prompt specific imaging studies to recognize suggestive signs of organ involvement. In this study, a case of a patient with ECD with representative progressive multisystemic involvement has been reported; although the final diagnosis was confirmed by histologic analysis, imaging studies with almost pathognomonic findings guided the diagnostic process and prompted different therapeutic approaches according to the localization of the disease.
authors.affiliation: Università Vita-Salute San Raffaele, School of Medicine, San Raffaele Scientific Institute, Milan, Italy. dellatorre.emanuel@hsr.it
authors.forename: Emanuel
authors.initials: E
authors.lastname: Della Torre
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Dagna
authors.forename: Paola
authors.initials: P
authors.lastname: Mapelli
authors.forename: Renata
authors.initials: R
authors.lastname: Mellone
authors.forename: Maria
authors.initials: M
authors.lastname: Grazia Sabbadini
mesh_terms: D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D015609:Organotechnetium Compounds; D014057:Tomography, X-Ray Computed
chemical_list: D015609:Organotechnetium Compounds