【摘要】 目的：分析总结Rosai-Dorfman病（RDD）的临床特点及其影像表现，以提高对该病的诊断。方法：回顾性分析17例经病理证实的RDD的临床及影像特征。男12例，女5例，年龄12～71岁。结果：病变位于头颈部9例，胸部6例，腹部1例及下肢1例。研究发现部分RDD的发病与IgG4等免疫相关疾病或淋巴瘤密切相关。影像表现为均匀的软组织密度/信号，无坏死及囊变，中度弥散受限及18F-FDG高摄取，其中中枢神经系统及非中枢神经系统RDD影像表现有所不同。另外发现3例罕见病例：颅内外非沟通性RDD，全身广泛性腺体及淋巴结受累的混合型RDD，及MR表现类似“肝细胞癌”的肝脏RDD。结论：RDD发病部位广泛且影像表现多样，了解其共同特点及不同之处，有助于加强对该病的诊断及鉴别诊断。更多还原

【Abstract】 Objective: To analyze and summarize the clinical features and imaging findings of Rosai-Dorfman disease(RDD) in order to improve the diagnosis of the disease. Methods: The clinical and imaging features of 17 cases of RDD confirmed by pathology were analyzed retrospectively. There were 12 males and 5 females, aged 12 to 71 years old. Results:The lesions were located in the head and neck(9 cases), chest(6 cases), abdomen(1 case) and lower extremity(1 case). We found that the pathogenesis of some RDD was closely related to IgG4 and other immune-related diseases or lymphoma. The imaging findings of RDD were homogeneous soft tissue density/signal, no necrosis and cystic degeneration, moderate diffusion restriction and high18F-FDG uptake. The imaging findings of RDD in the central nervous system and non-central nervous system were different. We identified 3 rare cases, intracranial-extracranial noncommunicating RDD, mixed RDD with systemic involvement of glands and lymph nodes, and RDD of the liver with MR appearance similar to “hepatocellular carcinoma”.Conclusion: RDD occurs in a wide range of sites and imaging manifestations vary. Understanding the common characteristics and differences of RDD is helpful to strengthen the diagnosis and differential diagnosis of this disease.更多还原