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@ARTICLE{Zhang:274467,
      author       = {G.-J. Zhang and X.-J. Ma$^*$ and Y.-P. Zhang and L.-F. Hao
                      and L. Wang and J.-T. Zhang and Z. Wu and D. Li},
      title        = {{S}urgical management and outcome of primary intracranial
                      {R}osai-{D}orfman disease: a single-institute experience and
                      pooled analysis of individual patient data.},
      journal      = {Neurosurgical review},
      volume       = {46},
      number       = {1},
      issn         = {0344-5607},
      address      = {New York},
      publisher    = {Springer},
      reportid     = {DKFZ-2023-00612},
      pages        = {76},
      year         = {2023},
      note         = {DKFZ-ZMBH Alliance},
      abstract     = {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.},
      keywords     = {Primary intracranial Rosai-Dorfman disease (Other) /
                      Radiotherapy (Other) / Steroid therapy (Other) / Surgical
                      resection (Other)},
      cin          = {A240},
      ddc          = {610},
      cid          = {I:(DE-He78)A240-20160331},
      pnm          = {311 - Zellbiologie und Tumorbiologie (POF4-311)},
      pid          = {G:(DE-HGF)POF4-311},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:36967440},
      doi          = {10.1007/s10143-023-01983-9},
      url          = {https://inrepo02.dkfz.de/record/274467},
}