Observational studyNeuro-oncology2025
Local tumor control and neurological outcomes after surgery for spinal hemangioblastomas in sporadic and von Hippel-Lindau disease: A multicenter study.
Observational study in Neuro-oncology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- Holocord syringomyelia associated with von Hippel-Lindau disease: a case series.Frontiers in surgery · 2026Article
- Von Hippel-Lindau syndrome presenting as a lateral ventricular space-occupying lesion: a case report.Frontiers in oncology · 2026Article
- Comparing laminectomy and unilateral hemilaminectomy in spinal hemangioblastoma resection: A multicenter study.Brain & spine · 2026Article
- Prognostic relevance of MIB-1 labeling index in VHL-associated and sporadic spinal hemangioblastomas: a subgroup analysis from a multicentric study.Acta neuropathologica communications · 2025Article
- Genetic Markers and Mutations in Primary Spinal Cord Tumors and Their Impact on Clinical Management.Brain sciences · 2025Review
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Authors and funding
37 authors.
Funding
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Abstract
backgroundSpinal 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.
methodsThis 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.
resultsComplete 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.
conclusionsComplete 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.
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