Rosai-Dorfman病的临床影像学特征

Clinical and imagingg characteristics of Rosai-Dorfman disease

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。

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 high ISF-FDG uptake.The imaging findings of RDD in the central nervous system and non-central nervous system were dfferent.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.