Department of Radiology, Ghent University Hospital and Ghent University, Ghent, Belgium.
Department of Oncology, Ghent University Hospital and Ghent University, Ghent, Belgium.
Skeletal Radiol. 2023 Aug;52(8):1605-1618. doi: 10.1007/s00256-022-04265-5. Epub 2023 Jan 5.
This is, to our knowledge, the first case report with in-depth analysis of bone marrow and bone lesions with diffusion-weighted imaging and dynamic contrast-enhanced MRI in Erdheim-Chester disease to date. We present a case of a 70-year-old woman who was referred for an X-ray of the pelvis, right femur and right knee after complaints of migratory arthralgia in hip and knee five months after an initial hip and knee trauma. Bone lesions on X-ray were identified. This case report highlights the strength and complementary use of modern multimodality multiparametric imaging techniques in the clinical radiological manifestations of Erdheim-Chester disease, in the differential diagnosis and in treatment response assessment, which is classically performed using FDG PET-CT. Erdheim-Chester disease is a rare form of non-Langerhans' cell histiocytosis, mainly affecting individuals in their fifth-seventh decade of life and without sex predominance. Apart from the typical bilateral symmetric lesions in long bone diaphyseal and metaphyseal regions and classically sparing the epiphyses, this multisystemic disease causes significant morbidity by infiltrating critical organs (the central nervous system, cardiovascular system, retroperitoneum, lungs and skin). With non-traumatic bone pain being the most common complaint, Erdheim-Chester disease is diagnosed most often in an incidental setting on imaging. The imaging workup classically consists of a multimodality approach using conventional radiography, CT, MRI, bone scintigraphy and FDG PET-CT. This case report extends this evaluation with diffusion-weighted imaging and dynamic contrast-enhanced imaging techniques.
据我们所知,这是迄今为止首例深入分析骨髓和骨病变的病例报告,同时使用了弥散加权成像和动态对比增强 MRI。我们报告了一例 70 岁女性的病例,她在初次髋关节和膝关节创伤后 5 个月出现髋关节和膝关节游走性关节炎的症状,随后接受了骨盆、右股骨和右膝关节的 X 射线检查。X 射线检查发现了骨病变。本病例报告强调了现代多模态多参数成像技术在 Erdheim-Chester 病的临床放射学表现、鉴别诊断和治疗反应评估中的优势和互补性,这些在经典的 FDG PET-CT 中进行。Erdheim-Chester 病是一种罕见的非朗格汉斯细胞组织细胞增生症,主要影响 50 至 70 岁的个体,无性别优势。除了典型的双侧对称长骨干骺端和干骺端区域病变,经典地避开骺端外,这种多系统疾病通过浸润重要器官(中枢神经系统、心血管系统、腹膜后、肺和皮肤)导致显著的发病率。非创伤性骨痛是最常见的主诉, Erdheim-Chester 病最常在影像学检查中偶然发现。影像学检查通常采用多模态方法,包括常规放射摄影、CT、MRI、骨闪烁扫描和 FDG PET-CT。本病例报告扩展了这种评估,使用了弥散加权成像和动态对比增强成像技术。