title: Erdheim-Chester disease: clinical and radiological findings.
abstract: OBJECTIVE
  The authors retrospectively reviewed six cases of histologically proven Erdheim-Chester disease (ECD) to evaluate organ involvement and clinical and radiological findings.
  
  
  METHODS
  Through a search of the pathology databases of four Italian hospitals, we identified six men (mean age, 56 years) with a histological diagnosis of ECD. Histology was performed on retroperitoneal or pulmonary biopsy, depending on disease involvement on imaging. Patients underwent plain radiography of the lower limbs and chest, total-body computed tomography (CT) and bone scintigraphy. Magnetic resonance (MR) imaging was performed in two patients to evaluate the lower limbs and in one patient to study the brain, the chest and the abdomen.
  
  
  RESULTS
  Clinical manifestations included dyspnoea (n=2), hydronephrosis (n=2) and bone pain (n=1). Bilateral symmetrical osteosclerosis of the metaphyseal and diaphyseal portions of the lower-limb long bones was present in five patients. Imaging studies revealed extraskeletal manifestations in all patients, including involvement of the retroperitoneal space (n=4), the lung (n=4) and the heart (n=2).
  
  
  CONCLUSIONS
  ECD is a multiorgan disease that displays constant involvement of the bones and retroperitoneum; in particular, of the perirenal fat. Although the diagnosis of ECD is histological, imaging can raise suspicion and help to establish a presumptive diagnosis.
authors.affiliation: Dipartimento di Scienze Cliniche, Sezione di Scienze Radiologiche, Università degli Studi di Parma, Parma, Italy. massimo.defilippo@unipr.it
authors.forename: M
authors.initials: M
authors.lastname: De Filippo
authors.forename: A
authors.initials: A
authors.lastname: Ingegnoli
authors.forename: A
authors.initials: A
authors.lastname: Carloni
authors.forename: E
authors.initials: E
authors.lastname: Verardo
authors.forename: N
authors.initials: N
authors.lastname: Sverzellati
authors.forename: M
authors.initials: M
authors.lastname: Onniboni
authors.forename: A
authors.initials: A
authors.lastname: Corsi
authors.forename: S
authors.initials: S
authors.lastname: Tomassetti
authors.forename: M
authors.initials: M
authors.lastname: Mazzei
authors.forename: L
authors.initials: L
authors.lastname: Volterrani
authors.forename: V
authors.initials: V
authors.lastname: Poletti
authors.forename: M
authors.initials: M
authors.lastname: Zompatori
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D004417:Dyspnea / Q000209:etiology; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000000981:diagnostic imaging; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology; D007558:Italy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000209:etiology; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed