Department of Radiology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, USA.
Br J Radiol. 2019 Dec;92(1104):20190473. doi: 10.1259/bjr.20190473. Epub 2019 Aug 14.
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis which can have a broad range of clinical and radiological presentations. Typically, ECD affects multiple organ systems, with skeletal involvement present in almost all ECD patients and cardiothoracic manifestations in more than half. Cardiac and thoracic involvement contributes significantly to morbidity and mortality in affected patients and may have prognostic implications. The diagnosis of ECD can be challenging due to its rarity and similarity to other systemic disease processes. Although the diagnosis can be suggested on imaging, histopathology and immunohistochemistry are required for confirmation. We describe the multimodal imaging features of mediastinal, cardiac, pleural and lung parenchymal ECD. This review identifies the most common radiological manifestations of cardiac and thoracic ECD on contrast-enhanced CT, fluorine-fludeoxyglucose positron emission tomography/CT and cardiac MRI, and highlights the role of these cross-sectional techniques in disease diagnosis.
厄尔-道伊姆-切斯特病（ECD）是一种罕见的非朗格汉斯细胞组织细胞增生症，其临床表现和影像学表现广泛。通常，ECD 影响多个器官系统，骨骼受累几乎见于所有 ECD 患者，且半数以上患者存在心胸表现。心脏和胸部受累显著增加了受累患者的发病率和死亡率，并可能具有预后意义。由于其罕见性和与其他系统性疾病过程的相似性，ECD 的诊断具有挑战性。尽管影像学检查可提示诊断，但组织病理学和免疫组织化学检查仍为确诊所必需。我们描述了纵隔、心脏、胸膜和肺实质 ECD 的多模态影像学特征。本综述确定了心脏和胸部 ECD 在增强 CT、氟脱氧葡萄糖正电子发射断层扫描/CT 和心脏 MRI 上最常见的放射学表现，并强调了这些横断面技术在疾病诊断中的作用。
