Local tumor control and neurological outcomes after surgery for spinal hemangioblastomas in sporadic and Von-Hippel-Lindau Disease: A multicenter study.

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 (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.

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.