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

IF 13.4 1区医学 Q1 CLINICAL NEUROLOGY

Neuro-oncology Pub Date : 2025-07-30 DOI:10.1093/neuonc/noaf041

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, Erdem Güresir

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引用次数: 0

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.

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散发性和Von-Hippel-Lindau病脊柱血管母细胞瘤手术后局部肿瘤控制和神经预后：一项多中心研究

背景：脊髓血管母细胞瘤（sHBs）是一种罕见的血管性肿瘤，具有重要的神经学意义。由于复发和功能下降，他们的管理，特别是在von Hippel-Lindau （VHL）疾病中，仍然具有挑战性。及时识别和干预对获得最佳结果至关重要。方法：这项国际多中心回顾性队列研究包括来自13个神经肿瘤中心的357例患者（199例与vhl相关，158例散发）。分析临床和影像学数据，使用改良麦考密克量表（mMCS）评估12个月时的无进展生存期（PFS）和功能结局。二级分析确定了与sHBs中VHL疾病相关的因素。结果：87.7%的病例完全切除（CR），导致72个月时PFS显著改善(散发性：95.1%，vhl相关：91.1%；Hr: 0.18, 95%ci: 0.08-0.4)。多变量分析确定了12个月时不良预后的预测因素：术前mMCS≥2 （OR: 5.17, p=0.008）、髓内肿瘤位置（OR: 9.48, p=0.01）和术前出血（OR: 31.12, p=0.02）。与sHBs中VHL疾病独立相关的因素包括非宫颈肿瘤位置（OR: 2.08, p=0.004）、髓内生长（OR: 2.39）。结论：对于散发性和VHL相关sHBs，完全手术切除对于长期肿瘤控制和良好的功能预后至关重要。早期干预，特别是在症状轻微和进展性病例中，在神经功能恶化或出血之前进行干预，可优化康复。该研究是多中心国际环境中同类研究中规模最大的，为指导散发性和vhl相关sHBs的管理提供了强有力的证据。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuro-oncology 医学-临床神经学

CiteScore

27.20

自引率

6.30%

发文量

1434

审稿时长

3-8 weeks

期刊介绍： Neuro-Oncology, the official journal of the Society for Neuro-Oncology, has been published monthly since January 2010. Affiliated with the Japan Society for Neuro-Oncology and the European Association of Neuro-Oncology, it is a global leader in the field. The journal is committed to swiftly disseminating high-quality information across all areas of neuro-oncology. It features peer-reviewed articles, reviews, symposia on various topics, abstracts from annual meetings, and updates from neuro-oncology societies worldwide.