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@ARTICLE{Wach:298926,
      author       = {J. Wach and A. E. Basaran and M. Vychopen and T. Tihan and
                      M. Wostrack and V. M. Butenschoen and B. Meyer and S. Siller
                      and N. O. Schmidt and J. Onken and P. Vajkoczy and A. N.
                      Santos and L. Rauschenbach$^*$ and P. Dammann and U. Sure
                      and J.-H. Klingler and R. Doria-Medina and J. Beck and B.-I.
                      Blaß and C. J. Gizaw and R. Hohenhaus and S. Krieg and O.
                      T. Alhalabi and L. Klein and C. Thomé and N. Kögl and P.
                      Kunert and T. Czernicki and T. Pantel and M. Middelkamp and
                      S. O. Eicker and A. H. Kattaa and D. J. Park and S. D. Chang
                      and F. Kilinc and M. Czabanka and E. Güresir},
      title        = {{L}ocal tumor control and neurological outcomes after
                      surgery for spinal hemangioblastomas in sporadic and
                      {V}on-{H}ippel-{L}indau {D}isease: {A} multicenter study.},
      journal      = {Neuro-Oncology},
      volume       = {27},
      number       = {6},
      issn         = {1522-8517},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2025-00360},
      pages        = {1567–1578},
      year         = {2025},
      note         = {Volume 27, Issue 6, June 2025, Pages 1567–1578},
      abstract     = {Spinal hemangioblastomas (sHBs) are rare vascular tumors
                      with significant neurological implications. Their
                      management, particularly in von Hippel-Lindau (VHL) disease,
                      remains challenging due to recurrence and functional
                      decline. Timely identification and intervention are critical
                      for optimal outcomes.This international, multicenter
                      retrospective cohort study included 357 patients (199
                      VHL-associated, 158 sporadic) from 13 neuro-oncological
                      centers. Clinical and imaging data were analyzed to assess
                      progression-free survival (PFS) and functional outcomes
                      using the Modified McCormick Scale (mMCS) at 12 months.
                      Secondary analyses identified factors associated with VHL
                      disease in sHBs.Complete resection (CR) was achieved in
                      $87.7\%$ of cases, leading to significantly improved PFS at
                      72 months (sporadic: $95.1\%,$ VHL-associated: $91.1\%;$ HR:
                      0.18, $95\%CI:$ 0.08-0.4). Multivariable analysis identified
                      predictors of unfavorable outcomes at 12 months:
                      Preoperative mMCS ≥2 (OR: 5.17, p=0.008), intramedullary
                      tumor location (OR: 9.48, p=0.01), and preoperative bleeding
                      (OR: 31.12, p=0.02). Factors independently associated with
                      VHL disease in sHBs included non-cervical tumor location
                      (OR: 2.08, p=0.004), intramedullary growth (OR: 2.39,
                      p<0.001), and age <43 years (OR: 3.24, p<0.001). Functional
                      improvements were observed in most patients, particularly
                      those with sporadic sHBs.Complete surgical resection is
                      essential for long-term tumor control and favorable
                      functional outcomes in both sporadic and VHL-associated
                      sHBs. Early intervention, particularly in mild symptomatic
                      and progressive cases, before neurological deterioration or
                      hemorrhage, optimizes recovery. This study, the largest of
                      its kind in a multicentric international setting, provides
                      robust evidence to guide the management of both sporadic and
                      VHL-associated sHBs.},
      keywords     = {Complete resection (Other) / Multicenter study (Other) /
                      Neurological outcomes (Other) / Progression-free survival
                      (Other) / Spinal hemangioblastomas (Other) / Von
                      Hippel-Lindau disease (Other)},
      cin          = {ED01},
      ddc          = {610},
      cid          = {I:(DE-He78)ED01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39950840},
      doi          = {10.1093/neuonc/noaf041},
      url          = {https://inrepo02.dkfz.de/record/298926},
}