Kamaleshwaran Koramadai Karuppusamy, Ramkumar Elumalai
Department of Nuclear Medicine, PET/CT and Radionuclide Therapy, Kovai Medical Center and Hospital Limited, Coimbatore, Tamil Nadu, India.
Indian J Nucl Med. 2023 Oct-Dec;38(4):381-383. doi: 10.4103/ijnm.ijnm_63_23. Epub 2023 Dec 20.
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.
厄尔德海姆-切斯特病(ECD)是一种可累及多个器官的系统性组织细胞增多症,严重程度从隐匿性到危及生命不等。1930年,威廉·切斯特在与奥地利病理学家雅各布·厄尔德海姆合作后首次描述了这种疾病。即便在今天,鉴于ECD的罕见性和表现多样,正确诊断该病通常仍需数年时间。我们报告一例63岁女性,因出现多发性脑病变而接受氟-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灶,结果显示右枕叶脑病变、右眼眶病变及主动脉弓周围软组织(主动脉包绕)代谢增高,脑病变的最终组织病理学检查确诊为组织细胞增多症ECD。