脊柱Rosai-Dorfman病的诊断和治疗:系统性文献综述

Diagnosis and Treatment of Rosai-Dorfman Disease of the Spine

目的:通过回顾并分析文献,总结脊柱Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床特点和诊治现状。方法:回顾性收集2010年至今的脊柱RDD的病案报告,采用系统性综述的方式,分别以"Rosai-Dorfman disease, spine"、"Rosai-Dorfman disease,central nervous system"、"Rosai-Dorfman病、脊柱"为关键词搜索中文和英文全文数据库。经筛选共纳入文献43篇,合计患者52例。确立研究内容后,研读文献并提取相应信息,归纳后录入相应条目,即构建脊柱RDD数据库,重点研究和报告脊柱RDD的临床特点、诊疗现状和预后情况,并运用统计学方式对数据进行分析。结果:纳入文献共报告52例患者资料,年龄为(32.1±17.1)岁(范围6~76岁),男女比例为1.9∶1;中位随访时间为19.9个月。其中41例(78.8%)以脊柱相关症状起病,合并无痛性淋巴结肿大者为11.5%,合并其他器官受累者为36.5%,实验室检查结果异常者为23.1%。病灶节段以颈椎(43.1%)、胸椎(39.2%)为主。有53.2%的病灶起源于硬脊膜,骨、脊髓实质分别为17.0%、10.6%。手术为主要的治疗方法(83.7%),其中病灶全切者占手术病例的46.3%。放疗、化疗或激素治疗仅应用于少数病例(10.2%)。极少数患者有自愈倾向(2.0%)。脊柱RDD术后复发或再发比例达22.0%。结论:脊柱RDD为临床罕见病,并无特征性的临床和影像学表现,有多器官侵犯、复发或再发的潜力;该病的诊断依靠术后病理,治疗以手术为主,其他辅助治疗的疗效并不确切,无症状者可给予随访观察。

Objective to review the clinical features,diagnosis and treatment of spinal Rosai-Dorfman disease(RDD).Methods we conducted a systemic review and collected the cases reported from 2010.The key words were Rosai-Dorfman disease,spine/central nervous system.We screened both English and Chinese database.There were 43 reports finally included in the study,containing 52 cases.We distracted the information of interest and,subsequently,analyzed the harvested data using specific statistical software packages.The study focused on the summary and description of the clinical features,diagnosis and treatment and prognosis of spinal RDD.Results The included articles reported 52 cases.The average age was 32.1±17.1 years(ranging from 6 to 76 years old).The ratio of male to female was 1.9/1.The median follow-up period was 19.9 months.The initial symptoms of 41 patients(78.8%)were spinal lesion-related.The cases with painless lymph nodes enlargement,other organ lesions and abnormal lab tests were 11.5%,36.5%and 23.1%,respectively.The frequent infringed segments were cervical(43.1%)and thoracic(39.2%)spine.53.2%lesions were dura-based,while 17.0%and 10.6%for bone and cord,respectively.Surgery was the mainstream of the treatment armamentarium,composing 83.7%cases,among which 46.3%underwent total resection.Cases only treated with radiotherapy,chemotherapy and steroids were 10.2%.Very Few cases remitted spontaneously(2.0%).The risk of recurrence and occurrence at other vertebral levels was 22.0%.Conclusion It is rare for spinal involvement of RDD.This entity has no pathognomonic clinical and imaging features.RDD has a tendency of multi-organ involvement and recurrence.Surgery remains the mainstay of the treatment,but the efficacy of other adjuvant therapies is not sure.A wait and watch strategy is employed for asymptomatic patients.