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Year : 2018  |  Volume : 131  |  Issue : 4  |  Page : 440-447

A Study on Clinical Characteristics and Magnetic Resonance Imaging Manifestations on Systemic Rosai-Dorfman Disease

Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China

Correspondence Address:
Dr. Jing-Liang Cheng
Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe Road, Zhengzhou, Henan 450000
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0366-6999.225053

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Background: Rosai-Dorfman disease (RDD) is typically characterized by painless bilateral and symmetrical cervical lymphadenopathy, with associated fever and leukocytosis. The aim of the current study was to summarize the clinical features and imaging characteristics of RDD, in an effort to improve its diagnostic accuracy. Methods: The study was analyzed from 32 patients between January 2011 and December 2017; of these, 16 patients had pathologically diagnosed RDD, eight had pathologically diagnosed meningioma, and eight pathologically diagnosed lymphoma. All patients underwent computed tomography and magnetic resonance imaging (MRI). Clinical features and imaging characteristics of RDD were analyzed retrospectively. The mean apparent diffusion coefficient (ADC) values of lesions at different sites were measured, and one-way analysis of variance and the least significant difference t-test were used to compare the differences between groups and draw receiver operating characteristic curves. The tumors were excised for biopsy and analyzed using immunohistochemistry. Results: The mean ADCs were (0.81 ± 0.10) × 10−3 mm2/s for intercranial RDD, (0.73 ± 0.05) × 10−3 mm2/s for nasopharyngeal RDD, (0.74 ± 0.11) × 10−3 mm2/s for bone RDD, and (0.71 ± 0.04) × 10−3 mm2/s for soft-tissue RDD. The optimum ADC to distinguish intracranial RDD from lymphoma was 0.79 × 10−3 mm2/s (62.5% sensitivity and 100% specificity) and to distinguish meningioma from intracranial RDD was 0.92 × 10−3 mm2/s (62.5% sensitivity and 100% specificity). Levels of C-reactive protein, erythrocyte sediment rate and D-dimer were significantly elevated (81%, 87%, and 75%, respectively). On immunohistochemistry, RDD was positive for both S-100 and CD68 proteins but negative for CD1a. Conclusions: Conventional MRI, combined with diffusion-weighted imaging and ADC mapping, is an important diagnostic tool in evaluating RDD patients. An accurate diagnosis of RDD should consider the clinical features, imaging characteristics, and the pathological findings.


 Abstract in Chinese



背景: Rosai-Dorfman病(RDD)是以无痛, 对称性, 双侧颈淋巴结增生为典型表现的疾病, 常伴随发热和白细胞增多。 本研究总结了16例RDD的临床病理和影像学特征, 以提高诊断的准确性。

方法: 回顾分析了2011年1月至2017年12月就诊于我院的32名患者, 术后免疫组化及病理证实16例为RDD, 8例为脑膜瘤, 8例为淋巴瘤, 分析RDD的临床及影像特征。 患者均进行了CT及MRI扫描, 测量不同部位疾病的 表观弥散系数 (ADC)值, 使用单因素方差分析比较各组均值, LSD-t检验比较两两差异, 受试者工作特征 (ROC)曲线预测最佳阈值。

结果: 颅内RDD的平均ADC值是 (0.81  0.10) × 10−3 mm2/s, 鼻咽部RDD的平均ADC值是 (0.73 ± 0.05 )× 10−3mm2/s,骨RDD的平均ADC值是 (0.74  0.11) × 10−3 mm2/s, 软组织RDD的平均ADC值是 (0.71  0.04) × 10−3 mm2/s。 区别颅内RDD与淋巴瘤的最佳ADC阈值是 0.79 × 10−3 mm2/s (62.5% 敏感性, 100%特异性), 区别颅内RDD与脑膜瘤的最佳ADC阈值是0.92 × 10−3 mm2/s (62.5%敏感性, 100%特异性)。 RDD的C-反应蛋白, 血沉和D-二聚体都显著升高 (分别为81%, 87%和75%)。 RDD免疫组化结果表现为S-100 和 CD68为阳性, 而CD1a为阴性。

结论: 传统MRI, 结合弥散加权成像(DWI)及ADC值, 可以作为评估RDD患者的重要方法。 RDD的准确诊断需考虑临床特征, 影像特征及病理结果。

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