PDF(Pubmed)
Abstract：
Erdheim-Chester disease (ECD) is an uncommon non-Langerhan cell histiocytosis that affects multiple systems and most commonly involves the bones. A 34-year-old patient with a three-month history of progressive dysphagia, underwent the gastroscopy which revealed esophageal mucosal constriction 34 cm from the incisor and external pressure stenosis. Enhanced computed tomography (CT), magnetic resonance imaging (MRI), and 2-[fluorine-18] fluoro-2-deoxy-d-glucose (18F-FDG) positron emission tomography (PET)/CT findings confirmed diffuse soft-tissue infiltration filled periesophageal space and excluded lesions involving the bone and other organs within the scanning range. The patient was later diagnosed with esophageal involvement of ECD by thoracotomy surgery and paraesophageal soft tissue biopsy. Progressive dysphagia, a rare clinical manifestation of ECD, has been reported in only two cases. It is the first demonstration of MRI and PET/CT imaging findings of ECD esophageal invasion as far as we known. Through the comparison of multiple images, we have a preliminary recognition of characteristic radiological features of gastrointestinal infiltration in ECD.
摘要：
Erdheim-Chester病（ECD）是一种罕见的非朗格汉细胞组织细胞增生症，影响多个系统，最常见的是骨骼。一名34岁的患者，有三个月的进行性吞咽困难病史，行胃镜检查，发现距切牙34厘米的食管粘膜狭窄和外部压力狭窄。增强计算机断层扫描（CT），磁共振成像（MRI），和2-[氟-18]氟-2-脱氧-d-葡萄糖（18F-FDG）正电子发射断层扫描（PET）/CT检查结果证实弥漫性软组织浸润充满食管周围间隙，并排除了扫描范围内涉及骨骼和其他器官的病变。该患者后来通过开胸手术和食管旁软组织活检被诊断为食管累及ECD。进行性吞咽困难,一种罕见的ECD临床表现，只报告了两起案件。据我们所知，这是ECD食管浸润的MRI和PET/CT成像发现的首次证明。通过多幅图像的比较，我们对ECD中胃肠道浸润的特征性放射学特征有了初步认识。
