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Local tumor control and neurological outcomes after surgery for spinal hemangioblastomas in sporadic and von Hippel-Lindau disease: a multicenter study

BACKGROUND: 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 op...

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Main Authors: Wach, Johannes (Author) , Basaran, Alim Emre (Author) , Vychopen, Martin (Author) , Tihan, Tarik (Author) , Wostrack, Maria (Author) , Butenschoen, Vicki M. (Author) , Meyer, Bernhard (Author) , Siller, Sebastian (Author) , Schmidt, Nils Ole (Author) , Onken, Julia (Author) , Vajkoczy, Peter (Author) , Santos, Alejandro N. (Author) , Rauschenbach, Laurèl (Author) , Dammann, Philipp (Author) , Sure, Ulrich (Author) , Klingler, Jan-Helge (Author) , Doria-Medina, Roberto (Author) , Beck, Jürgen (Author) , Blaß, Bianca-Ioana (Author) , Gizaw, Christine Julia (Author) , Hohenhaus, Romina (Author) , Krieg, Sandro (Author) , Alhalabi, Obada (Author) , Klein, Lukas (Author) , Thomé, Claudius (Author) , Kögl, Nikolaus (Author) , Kunert, Przemyslaw (Author) , Czernicki, Tomasz (Author) , Pantel, Tobias (Author) , Middelkamp, Maximilian (Author) , Eicker, Sven Oliver (Author) , Kattaa, Ahed H. (Author) , Park, David J. (Author) , Chang, Steven D. (Author) , Kilinc, Fatma (Author) , Czabanka, Marcus (Author) , Güresir, Erdem (Author)
Format: Article (Journal)
Language:English
Published: 15 February 2025
In: Neuro-Oncology
Year: 2025, Volume: 27, Issue: 6, Pages: 1567-1578
ISSN:1523-5866
DOI:10.1093/neuonc/noaf041
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1093/neuonc/noaf041
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Author Notes:Johannes Wach, Alim Emre Basaran, Martin Vychopen, Tarik Tihan, Maria Wostrack, Vicki M. Butenschoen, Bernhard Meyer, Sebastian Siller, Nils Ole Schmidt, Julia Onken, Peter Vajkoczy, Alejandro N. Santos, Laurèl Rauschenbach, Philipp Dammann, Ulrich Sure, Jan-Helge Klingler, Roberto Doria-Medina, Jürgen Beck, Bianca-Ioana Blaß, Christine Julia Gizaw, Romina Hohenhaus, Sandro Krieg, Obada T. Alhalabi, Lukas Klein, Claudius Thomé, Nikolaus Kögl, Przemyslaw Kunert, Tomasz Czernicki, Tobias Pantel, Maximilian Middelkamp, Sven Oliver Eicker, Ahed H. Kattaa, David J. Park, Steven D. Chang, Fatma Kilinc, Marcus Czabanka, and Erdem Güresir
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Summary:BACKGROUND: 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. - METHODS: 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. - RESULTS: Complete resection was achieved in 87.7% of cases, leading to significantly improved PFS at 72 months (sporadic: 95.1%, VHL-associated: 91.1%; hazard ratio: 0.18, 95% CI: 0.08-0.4). Multivariable analysis identified predictors of unfavorable outcomes at 12 months: preoperative mMCS ≥2 (odds ratio [OR]: 5.17, P = .008), intramedullary tumor location (OR: 9.48, P = .01), and preoperative bleeding (OR: 31.12, P = .02). Factors independently associated with VHL disease in sHBs included non-cervical tumor location (OR: 2.08, P = .004), intramedullary growth (OR: 2.39, P < .001), and age <43 years (OR: 3.24, P < .001). Functional improvements were observed in most patients, particularly those with sporadic sHBs. - CONCLUSIONS: 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.
Item Description:Gesehen am 24.03.2026
Physical Description:Online Resource
ISSN:1523-5866
DOI:10.1093/neuonc/noaf041

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