Local tumor control and neurological outcomes after surgery for spinal hemangioblastomas in sporadic and von Hippel-Lindau disease: A multicenter study.
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Wach Johannes, Basaran Alim Emre, Vychopen Martin, Tihan Tarik, Wostrack Maria, Butenschoen Vicki M, Meyer Bernhard, Siller Sebastian, Schmidt Nils Ole, Onken Julia, Vajkoczy Peter, Santos Alejandro N, Rauschenbach Laurèl, Dammann Philipp, Sure Ulrich, Klingler Jan-Helge, Doria-Medina Roberto, Beck Jürgen, Blaß Bianca-Ioana, Gizaw Christine Julia, Hohenhaus Romina, Krieg Sandro, Alhalabi Obada T, Klein Lukas, Thomé Claudius, Kögl Nikolaus, Kunert Przemyslaw, Czernicki Tomasz, Pantel Tobias, Middelkamp Maximilian, Eicker Sven Oliver, Kattaa Ahed H, Park David J, Chang Steven D, Kilinc Fatma, Czabanka Marcus, Güresir Erdem
Аннотация
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.