Wach, Johannes and Basaran, Alim Emre and Vychopen, Martin and Tihan, Tarik and Wostrack, Maria and Butenschoen, Vicki M. and Meyer, Bernhard and Siller, Sebastian and Schmidt, Nils Ole and Onken, Julia and Vajkoczy, Peter and Santos, Alejandro N. and Rauschenbach, Laurel and Dammann, Philipp and Sure, Ulrich and Klingler, Jan-Helge and Doria-Medina, Roberto and Beck, Juergen and Blass, Bianca-Ioana and Gizaw, Christine Julia and Hohenhaus, Romina and Krieg, Sandro and Alhalabi, Obada T. and Klein, Lukas and Thome, Claudius and Koegl, Nikolaus and Kunert, Przemyslaw and Czernicki, Tomasz and Pantel, Tobias and Middelkamp, Maximilian and Eicker, Sven Oliver and Kattaa, Ahed H. and Park, David J. and Chang, Steven D. and Kilinc, Fatma and Czabanka, Marcus and Gueresir, Erdem (2025) Local tumor control and neurological outcomes after surgery for spinal hemangioblastomas in sporadic and von Hippel-Lindau disease: A multicenter study. NEURO-ONCOLOGY, 27 (6). pp. 1567-1578. ISSN 1522-8517, 1523-5866
Full text not available from this repository. (Request a copy)Abstract
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 Type: | Article |
|---|---|
| Uncontrolled Keywords: | CENTRAL-NERVOUS-SYSTEM; CORD HEMANGIOBLASTOMAS; SURGICAL-MANAGEMENT; RESECTION; complete resection; multicenter study; neurological outcomes; progression-free survival; spinal hemangioblastomas; von Hippel-Lindau disease |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurochirurgie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 01 Apr 2026 05:50 |
| Last Modified: | 01 Apr 2026 05:50 |
| URI: | https://pred.uni-regensburg.de/id/eprint/67774 |
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