软组织Rosai-Dorfman病(附1例报告及文献复习)

Primary Soft Tissue Rosai-Dorfman Disease,Provided An Case Report and Reviewed the Relative Literature

目的:探讨1例原发性软组织Rosai-Dorfman病(RDD)的临床病理特征及MRI表现,强调在评估软组织恶性肿瘤时应将该病纳入鉴别诊断,以减少误诊率。方法:回顾性分析经手术和病理证实的1例软组织RDD的临床病理特征及MRI表现,并复习相关文献。结果:患者临床表现为左上臂肿物10余年明显增大3月余。MRI示左上臂皮下软组织肿块大小约7.5cm×4.5cm×12cm,T1WI呈稍低信号,T2WI呈稍高信号,DWI(弥散加权成像)呈高信号,显著强化,内见多发线状分隔,肿块外侧边界毛糙、深入皮肤深层。CE-MRA显示肿块内及周边有明显增多血管网,MRI拟诊软组织肉瘤。术前穿刺活检提示炎性病变,完整切除术后病理证实为软组织RosaiDorfman病。结论:单纯性结外型软组织RDD罕见,临床和影像学表现往往类似恶性肿瘤,确诊难度较大,需结合临床、影像以及病理,MRI有助于帮助排除其它可能的诊断以及有效地评估本病的进展程度。

Objective： To investigate the clinicopathologieal features and MRI findings of 1 case of primary soft tissue Rosai - Dorfman disease in order to emphasized that the disease should be included in the differential diagnosis to evaluate the misdiagnosis rate when assessing soft tissue malignancies. Methods： MRI appearance and clinicopathologie features in a case of histopathology confirmed Rosai - Dorfman disease were retrospectively analyzed and the related literatures were reviewed. Results： The patient complained that a mass grown in her left upper arm more than 10 years and increased significantly re- cently. MRI showed the mass with some linear separations and a rough border, size of about 7. 5em x4. 5cm x 12em, of moderate T1WI signal and slightly higher T2WI signal, and high signal of DWI. Contrast - enhanced MR/ showed lesions were significantly enhanced, The CE - MRA showed it is rich of blood vessels, diagnosis of soft tissue sarcoma is accordingly made. Preoperative biopsy showed inflammatory lesions, complete resection of the pathology confirmed as soft tissue Rosai - Dorfman disease. Conclusion： soft tissue RDD is rare and difficult to diagnose, the clinical and radiographic findings are of- ten similar to malignancies. MRI could help to eliminate other possible diagnoses and effectively evaluate the progress of the disease.