PURPOSE: The authors retrospectively reviewed six cases of histologically proven Erdheim-Chester disease (ECD) to evaluate organ involvement and clinical and radiological findings. MATERIALS AND 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.

De Filippo, M., Ingegnoli, A., Carloni, A., Verardo, E., Sverzellati, N., Onniboni, M., et al. (2009). Erdheim-Chester disease: clinical and radiological findings. LA RADIOLOGIA MEDICA, 114(8), 1319-1329 [10.1007/s11547-009-0473-8].

Erdheim-Chester disease: clinical and radiological findings.

MAZZEI, MARIA ANTONIETTA;VOLTERRANI, LUCA;
2009

Abstract

PURPOSE: The authors retrospectively reviewed six cases of histologically proven Erdheim-Chester disease (ECD) to evaluate organ involvement and clinical and radiological findings. MATERIALS AND 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.
File in questo prodotto:
File Dimensione Formato  
chester.pdf

non disponibili

Tipologia: Post-print
Licenza: NON PUBBLICO - Accesso privato/ristretto
Dimensione 912.52 kB
Formato Adobe PDF
912.52 kB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11365/24349
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo