Multisystemic Imaging Findings in Chinese Patients With Erdheim-Chester Disease

OBJECTIVE. Radiology is essential for diagnosing and managing Erdheim-Chester disease (ECD), a rare multisystemic non-Langerhans cell histiocytic neoplasm. This study aimed to systemically investigate imaging characteristics of patients with ECD. MATERIALS AND METHODS. Radiographic, CT, and MR images of 28 Chinese patients (14 male, 14 female; 5-65 years old) diagnosed with ECD on histopathology at our medical center between January 2014 and January 2019 were retrospectively analyzed. Two radiologists analyzed the images in consensus and recorded the imaging manifestations qualitatively. RESULTS. Seventeen of the 23 patients (73.9%) who underwent CNS evaluation had findings positive for ECD including supratentorial and infratentorial regions; infiltration of the corpus callosum, choroid plexus, pineal body, and walls of cerebral arteries; and brain nodules mimicking metastases. In the facial and orbital regions, postorbital masses (34.8%) and masses in the nasal sinuses and mastoid processes were noted. Moreover, bone involvement was found in 27 (96.4%) patients, with lower extremities most commonly affected (85.7%); pathologic fracture caused by bone involvement was also observed. On chest CT, pulmonary parenchyma, mesenchyme, pleura, and mediastinum were seen to be infiltrated by ECD (57.1%). Half (50.0%) of the 28 patients had cardiovascular involvement with infiltration of the aorta and its branches, coronary arteries, or branches of the superior mesenteric artery resulting in stenosis and ischemic symptoms. In addition to retroperitoneal involvement in 13 (46.4%) patients, a presacral mass wrapping bilateral pelvic ureters and thickening of the mesentery, peritoneum, and omentum were also detected. CONCLUSION. Patients with ECD should be evaluated systemically, because involvement of vital organs could be lethal.