| 引用本文: | 吴月琴,宋瑞瑞,张勇.13例Rosai-Dorfman病临床、影像与病理学特征分析及误诊对策[J].中国临床新医学,,():-. |
| 吴月琴.13例Rosai-Dorfman病临床、影像与病理学特征分析及误诊对策[J].中国临床新医学,,():-. |
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| 13例Rosai-Dorfman病临床、影像与病理学特征分析及误诊对策 |
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吴月琴1, 宋瑞瑞2, 张勇2
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1.山西省肿瘤医院、中国医学科 学院肿瘤医院山西医院、山西医科大学附属肿瘤医院;2.山西省肿瘤医院
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| 摘要: |
| 目的:基于13例确诊病例,系统分析Rosai-Dorfman病(RDD)的多模态特征与误诊模式,旨在构建以减少误诊为目标的临床诊断路径,为早期识别与准确诊断提供参考。方法:回顾性分析2016年1月至2025年9月经病理确诊的13例RDD患者的临床、影像及病理资料。所有患者均接受超声、计算机断层摄影(CT)或磁共振成像(MRI)中的至少一种影像学检查。病理学分析包括苏木精-伊红(HE)染色观察组织形态及免疫组化检测(S100、CD68、CD1a等标志物)。同时,对2018年至2025年间发表的13篇文献(共62例RDD患者)进行系统性复习。结果:13例患者中男6例,女7例,中位年龄51岁。病变部位包括软组织(5例)、头部(4例)、淋巴结(3例)及肺部(1例)。临床表现因发生部位不同而表现不一。超声检查多显示不均匀回声伴血流信号；CT与MRI增强扫描以不均匀强化为主(仅各有1例均匀强化)。影像学表现缺乏特异性,常被拟诊为淋巴瘤(1例)、海绵状血管瘤(1例)、神经源性肿瘤(1例)、脂肪瘤(1例)、脑膜瘤(4例),另有5例影像学报告仅描述了占位性病变,而未给出确切诊断。镜下形态大致相同,可见显著增生的组织细胞,周边见大量淋巴细胞、浆细胞浸润,成片的组织细胞与成片的淋巴浆细胞呈交替排列的暗区和亮区分布,高倍镜下可见“伸入运动”,即组织细胞内含有大量完整的淋巴细胞。其中HE染色见“伸入运动”7例,漏诊率为46.15%；免疫组化显示S100(100%)阳性、CD68(100%)阳性,而CD1a均为阴性。13例患者均获得随访,随访时间为1-110个月,中位随访时间为33个月,所有患者均存活,其中复发3例。文献复习(2018-2025年)共纳入13篇文献62例患者,结果显示RDD好发于结外部位(91.94%),临床表现多样,影像学缺乏特异性,确诊依赖病理检查,治疗以手术为主,预后良好,少数病例可复发。结论:RDD临床表现不典型,影像学表现缺乏特异性,极易误诊。病理学检查是确诊的关键,其中组织细胞“伸入运动”及S100/CD68共阳性为诊断核心依据。建议建立以病理为核心的影像-病理多模态诊断路径,以提升诊断准确性。 |
| 关键词: Rosai-Dorfman病 影像学检查 病理学诊断 误诊 临床决策 |
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| Clinical, imaging and pathological features of Rosai-Dorfman disease: analysis of 13 cases and misdiagnosis strategy |
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吴月琴
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Shanxi Province Cancer Hospital/ Shanxi Hospital Affiliated to Cancer Hospital, Chinese Academy of Medical Sciences/Cancer Hospital Affiliated to Shanxi Medical University
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| Abstract: |
| Objective: Based on 13 confirmed cases, the multimodal characteristics and misdiagnosis patterns of Rosai-Dorfman disease (RDD) were systematically analyzed, aiming to build a clinical diagnosis path with the goal of reducing misdiagnosis and provide reference for early identification and accurate diagnosis. Methods: A retrospective analysis was conducted on the clinical, imaging, and pathological data of 13 patients with pathologically confirmed RDD between January 2016 and September 2025. All patients underwent at least one imaging examination, including ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI). Pathological analysis involved hematoxylin and eosin (HE) staining to observe histological morphology and immunohistochemical detection of markers such as S100, CD68, and CD1a. Results: Among the 13 patients, 6 were male and 7 were female, with a median age of 51 years. The anatomical distribution of lesions was as follows: soft tissue (5 cases), head (4 cases), lymph nodes (3 cases), and lung (1 case). Clinical manifestations varied significantly based on the anatomical location of involvement. Ultrasonography predominantly demonstrated heterogeneous echogenicity with detectable blood flow signals. Contrast-enhanced CT and MRI examinations primarily revealed heterogeneous enhancement patterns, with only one case in each modality demonstrating homogeneous enhancement. Imaging findings lacked specificity, and were often diagnosed as lymphoma (1 case), cavernous hemangioma (1 case), neurogenic tumor (1 case), lipoma (1 case), and meningioma (4 cases). In addition, imaging reports of 5 cases only described space-occupying lesions without giving a precise diagnosis. Histopathological examination demonstrated characteristic features: prominent histiocytic proliferation accompanied by substantial lymphocyte and plasma cell infiltration. The architectural pattern showed alternating dark and light zones composed of sheets of histiocytes and lymphoplasmacytic cells. High-power microscopy revealed emperipolesis, characterized by intact lymphocytes within histiocyte cytoplasm. Among these cases, emperipolesis was observed on HE staining in 7 cases, resulting in a missed diagnosis rate of 46.15%. Immunohistochemical analysis demonstrated consistent positivity for S100 (100%) and CD68 (100%), while CD1a was uniformly negative in all cases. Thirteen patients were followed up for a duration ranging from 1 to 110 months, with a median follow-up time of 33 months. All patients survived, among whom 3 experienced recurrence. Literature review of publications from 2019 to 2023, encompassing 13 studies and 62 cases, indicated that RDD predominantly involves extranodal sites (91.94%), presents with diverse clinical manifestations, lacks specific imaging characteristics, requires pathological confirmation for definitive diagnosis, is primarily managed surgically, and demonstrates favorable prognosis, although a minority of cases may recur. Conclusion: RDD exhibits non-specific clinical manifestations and lacks characteristic imaging features, making it highly susceptible to misdiagnosis. Pathological examination remains crucial for definitive diagnosis, with histiocytic "emperipolesis" and co-expression of S100/CD68 serving as the core diagnostic criteria. We recommend establishing a pathology-centered imaging-pathology multimodal diagnostic pathway to improve diagnostic accuracy. |
| Key words: Rosai-Dorfman disease, diagnostic imaging, pathological diagnosis, diagnostic errors, clinical decision-making |
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