Chiocchi Marcello, Luciano Alessandra, De Stasio Vincenzo, Pugliese Luca, Di Donna Carlo, Cerocchi Martina, Gigliotti Paola, Carini Alessandro, Chirico Flavia, Camedda Riccardo, Di Biagio Daniele, Sbordone Paolo Francesco, Garaci Francesco, Floris Roberto
Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy.
Department of Diagnostic Oncoematology and Nuclear Medicine, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy.
Radiol Case Rep. 2023 Mar 26;18(5):2047-2054. doi: 10.1016/j.radcr.2023.02.063. eCollection 2023 May.
Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report.
厄尔德海姆-切斯特病是一种累及多个系统的罕见非朗格汉斯细胞组织细胞增多症。关于这种疾病的成像能力的知识有限。我们报告了一例极其罕见的厄尔德海姆-切斯特病病例，患者为一名67岁男性，有多系统受累，包括心血管系统、骨骼、腹膜后（肾脏和肾上腺浸润）及神经系统。使用计算机断层扫描、磁共振成像、正电子发射断层扫描和骨闪烁显像等多模态成像方式对各个器官的受累情况进行了全面评估。骨活检确诊为厄尔德海姆-切斯特病。特别是当心脏和大脑受累时，厄尔德海姆-切斯特病是一种罕见且预后不良的疾病。了解厄尔德海姆-切斯特病的成像特征可能有助于理解当前病例报告中所述及讨论的该疾病累及多个器官的放射学结果。
