Zhang Gui-Jun, Ma Xiu-Jian, Zhang Ya-Ping, Hao Li-Fang, Wang Liang, Zhang Jun-Ting, Wu Zhen, Li Da
Department of Neurosurgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.
Division of Molecular Neurogenetics, German Cancer Research Center (DKFZ), DKFZ-ZMBH Alliance, Im Neuenheimer Feld 280, 69120, Heidelberg, Germany.
Neurosurg Rev. 2023 Mar 27;46(1):76. doi: 10.1007/s10143-023-01983-9.
Primary intracranial Rosai-Dorfman disease (PIRDD) is considered a nonmalignant nonneoplastic entity, and the outcome is unclear due to its rarity. The study aimed to elaborate the clinic-radiological features, treatment strategies, and progression-free survival (PFS) in patients with PIRDD. Patients with pathologically confirmed PIRDD in our institute were reviewed. Literature of PIRDD, updated until December 2019, was systematically searched in 7 databases (Embase, PubMed, Cochrane database, Web of Science, Wanfang Data Knowledge Service Platform, the VIP Chinese Science and Technology Periodical Database (VIP), and the China National Knowledge Infrastructure (CNKI)). These prior publication data were processed and used according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Clinical-radiological characteristics and adverse factors for PFS were evaluated in the pooled cohort. The pooled cohort of 124 cases (81 male and 43 female), with a mean age of 39.7 years, included 11 cases from our cohort and 113 cases from 80 prior studies. Twenty-nine patients (23.4%) had multiple lesions. Seventy-four patients (59.7%) experienced gross total resection (GTR), 50 patients (40.3%) had non-GTR, 15 patients (12.1%) received postoperative adjuvant radiation, and 23 patients (18.5%) received postoperative steroids. A multivariate Cox regression revealed that GTR (HR = 4.52; 95% CI 1.21-16.86; p = 0.025) significantly improved PFS, and multiple lesions (p = 0.060) tended to increase the hazard of recurrence. Neither radiation (p = 0.258) nor steroids (p = 0.386) were associated with PFS. The overall PFS at 3, 5, and 10 years in the pooled cohort was 88.4%, 79.4%, and 70.6%, respectively. The PFS at 5 and 10 years in patients with GTR was 85.4% and 85.4%, respectively, which was 71.5% and 35.8%, respectively, in patients without GTR. Gross total resection significantly improved PFS and was recommended for PIRDD. Radiation and steroids were sometimes empirically administered for residual, multiple, or recurrent PIRDD, but the effectiveness remained arguable and required further investigation.Systematic review registration number: CRD42020151294.
原发性颅内罗萨伊-多夫曼病（PIRDD）被认为是一种非恶性非肿瘤性疾病，由于其罕见性，其预后尚不清楚。本研究旨在阐述PIRDD患者的临床放射学特征、治疗策略和无进展生存期（PFS）。对我院经病理证实的PIRDD患者进行了回顾性研究。在7个数据库（Embase、PubMed、Cochrane数据库、Web of Science、万方数据知识服务平台、维普中文科技期刊数据库（VIP）和中国知网（CNKI））中系统检索了截至2019年12月的PIRDD文献。这些先前发表的数据根据系统评价和Meta分析的首选报告项目（PRISMA）指南进行处理和使用。在汇总队列中评估了PFS的临床放射学特征和不良因素。汇总队列包括124例患者（男性81例，女性43例），平均年龄39.7岁，其中11例来自我们的队列，113例来自80项先前的研究。29例患者（23.4%）有多处病变。74例患者（59.7%）实现了大体全切除（GTR），50例患者（40.3%）未实现GTR，15例患者（12.1%）接受了术后辅助放疗，23例患者（18.5%）接受了术后类固醇治疗。多因素Cox回归显示，GTR（HR = 4.52；95%CI 1.21 - 16.86；p = 0.025）显著改善了PFS，多处病变（p = 0.060）倾向于增加复发风险。放疗（p = 0.258）和类固醇治疗（p = 0.386）均与PFS无关。汇总队列3年、5年和10年的总体PFS分别为88.4%、79.4%和70.6%。GTR患者5年和10年的PFS分别为85.4%和85.4%，未实现GTR的患者分别为71.5%和35.8%。大体全切除显著改善了PFS，推荐用于PIRDD。对于残留、多发或复发的PIRDD，有时会经验性地给予放疗和类固醇治疗，但其有效性仍存在争议，需要进一步研究。系统评价注册号：CRD42020151294。
