White Theresa V, Silvester Nicholas C, Otero Hansel J
Des Moines University, Mayfield Heights, OH, USA.
Walter Reed National Military Medical Center, Bethesda, MD, USA.
Pediatr Radiol. 2016 Aug;46(9):1345-9. doi: 10.1007/s00247-016-3594-y. Epub 2016 Mar 30.
Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis with multi-organ infiltration that occurs mainly in adults. Pediatric cases are extremely rare. Here we report a case of multisystemic Erdheim-Chester disease in a 15-year-old boy with central nervous system involvement and skeletal findings. Positron emission tomography (PET) and MRI were used to demonstrate characteristic bilateral, symmetrical medullary involvement of the metadiaphyses of long bones in the absence of the classic sclerotic radiographic appearance. This illustrates the potential for earlier diagnosis and visualization of therapeutic response in children.
厄尔德海姆-切斯特病是一种罕见的非朗格汉斯细胞组织细胞增多症，可多器官浸润，主要发生于成人。儿科病例极为罕见。本文报告一例15岁男孩的多系统厄尔德海姆-切斯特病，该病例有中枢神经系统受累及骨骼表现。采用正电子发射断层扫描（PET）和磁共振成像（MRI）来显示长骨干骺端特征性的双侧对称性骨髓受累情况，而无典型的硬化性放射学表现。这说明了在儿童中早期诊断及观察治疗反应的可能性。
