头颈部结外Rosai-Dorfman病20例临床特征及疗效分析

Analysis of the clinical characteristics and efficacy of 20 cases of extranodal Rosai-Dorfman disease in head and neck

目的探讨头颈部淋巴结外Rosai-Dorfman病（RDD）的临床特征及不同治疗方法的疗效。方法回顾性分析2008年5月至2016年11月在首都医科大学附属北京同仁医院就诊的头颈部结外RDD患者的临床特点、病理学特征及疗效随访总结。结果20例头颈部结外RDD患者中女14例，男6例，发病年龄27～86岁，平均52.5岁。累及最多的器官为鼻部（14例），喉部（5例），其中5例为≥2个部位受累。确诊依靠病变部位活体组织学检查，病理组织学特征性表现为窦组织细胞增生，可见＂伸入现象＂ ；免疫组化染色显示窦组织细胞S-100和CD68阳性。13例确诊前有误诊经历，20例均行手术治疗，其中9例曾复发并多次手术，8例术后接受激素及免疫抑制剂治疗，至2017年2月所有病例均存活。结论头颈部结外RDD是一种少见的良性组织细胞增生性疾病，鼻部受累最多见，且易复发，诊断依靠特征性的病理组织学结果，手术仍是目前诊断治疗的首选方式，复发病例应用糖皮质激素及免疫抑制剂治疗有一定疗效。

ObjectiveTo analyze the clinical characteristics and efficacy of extranodal Rosai-Dorfman disease in head and neck. MethodsClinical and pathological features and follow-up of patients who were diagnosed as RDD at Beijing Tongren Hospital of Capital Medical University between May 2008 and November 2016 were analyzed retrospectively. ResultsThere were a total of 20 extranodal RDD patients in head and neck including 14 females and 6 males, aged from 27 to 86 years （mean 52.5 years）. Nose （14 cases） and throat （5 cases） were most commonly involved organs and 5 patients had 2 or more organs involved. Diagnosis depends on biopsy. Histopathologically, the lesions of RDD were characterized by the presence of large histiocytes with emperipolesis. The large sinus histiocytes were positive for S-100 and CD68 protein staining. Diagnostic Errors in 13 patients and operations in all patients were happened. More than 1 operation was performed in 9 recurrence patients. Eight patients were treated by Glucocorticoids and immunosuppressive agents. All the patients were alive until Feb 2017.ConclusionExtranodal RDD in head and neck is known as a rare benign idiopathic proliferative disease. Nose is the most common site involved, and recurrence easily. It is diagnosis depending on histopathological report. Operation is first choice in diagnosis and treatment now. Glucocorticoids and immunosuppressive agents are effective in recurrence patients.