PDF(Pubmed)
Abstract：
Erdheim-Chester disease (ECD) is a systemic histiocytosis that can involve several organs, with severity ranging from occult to life-threatening. The disease was first described by William Chester in 1930 after working with the Austrian pathologist Jakob Erdheim. Even today, a correct diagnosis of ECD often takes years, given the rarity and variable manifestations of ECD. We present a case of a 63-year-old female presenting with multiple brain lesions, sent for fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography to find primary, and it showed hypermetabolic right occipital brain lesion, right orbital lesion, and soft tissue around the arch of the aorta (coated aorta), and final histopathology of the brain lesion confirmed histiocytosis ECD.
摘要：
Erdheim-Chester病（ECD）是一种系统性组织细胞增生症，可累及多个器官，严重程度从隐匿到危及生命。在与奥地利病理学家JakobErdheim合作后，WilliamChester于1930年首次描述了这种疾病。即使在今天,ECD的正确诊断通常需要数年时间，考虑到ECD的稀有性和多变性表现。我们介绍了一个63岁的女性，患有多发性脑损伤，发送氟-18氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描以寻找原发性，它显示了右枕骨脑的高代谢病变，右眶病变,主动脉弓周围的软组织（涂层主动脉），最终脑部病变的组织病理学证实了组织细胞增生症的ECD。
