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对比 von Hippel-Lindau 病和散发性颅上血管母细胞瘤立体定向放射外科治疗结果的分析:一项多中心国际研究。

Comparative analysis of stereotactic radiosurgery outcomes for supratentorial hemangioblastomas in von hippel-lindau disease and sporadic cases: A multi-center international study.

机构信息

Department of Neurological Surgery, University of Virginia, Charlottesville, VA, USA.

Department of Neurological Surgery, University of Virginia, Charlottesville, VA, USA.

出版信息

J Clin Neurosci. 2024 Nov;129:110879. doi: 10.1016/j.jocn.2024.110879. Epub 2024 Oct 18.

Abstract

BACKGROUND

Hemangioblastomas (HBs) are rare, benign central nervous system (CNS) neoplasms that rarely occur in the supratentorial. Resection with the goal of gross total resection (GTR) is often considered the primary treatment. Stereotactic radiosurgery (SRS) has been utilized more commonly in unresectable or partially resected cases. In this study, we aimed to evaluate SRS's effectiveness and clinical outcomes in supratentorial HBs.

METHODS

A retrospective analysis of multi-centers from 1993 to 2022 was conducted on patients with supratentorial HB treated with SRS.

RESULTS

The study included 13 patients with 30 supratentorial HBs (24 von Hippel-Lindau (VHL), 6 sporadic). Median age was 26 years. Most tumors were solid (86.2 %). SRS was primary treatment in 86.7 % of cases and adjuvant in 13.3 %. VHL lesions were significantly smaller than sporadic ones (0.2 vs. 3.7 cc, p = 0.009). Median margin dose was higher in VHL cases (17 vs. 13.0 Gy, P = 0.031). For VHL cases, local control (LC) was 100 % at 6 months, 96 % (95 % CI: 88 %, 100 %) from 12 to 60 months post-SRS. For sporadic cases, LC was 100 % at 6 months, 80 % (95 % CI: 52 %, 100 %) from 12 to 60 months post-SRS (p = 0.39). No adverse radiation events or mortality occurred.

CONCLUSION

SRS demonstrated a promising role in the clinical course of supratentorial HBs. It can be considered an effective alternative to surgical resection and even a first-line therapeutic option in appropriately selected cases.

摘要

背景

血管母细胞瘤(HBs)是一种罕见的良性中枢神经系统(CNS)肿瘤,很少发生在幕上。以大体全切除(GTR)为目标的切除术通常被认为是主要治疗方法。立体定向放射外科(SRS)在无法切除或部分切除的病例中应用更为普遍。在这项研究中,我们旨在评估 SRS 在幕上 HBs 中的有效性和临床结果。

方法

对 1993 年至 2022 年多中心的 13 例幕上 HB 患者进行 SRS 治疗的回顾性分析。

结果

该研究包括 13 例患者的 30 个幕上 HB(24 例 von Hippel-Lindau [VHL],6 例散发性)。中位年龄为 26 岁。大多数肿瘤为实体性(86.2%)。SRS 是 86.7%病例的主要治疗方法,13.3%的病例为辅助治疗。VHL 病变明显小于散发性病变(0.2 比 3.7 cc,p=0.009)。VHL 病例的中位边缘剂量较高(17 比 13.0 Gy,P=0.031)。对于 VHL 病例,SRS 后 6 个月时局部控制(LC)率为 100%,12-60 个月时为 96%(95%可信区间:88%,100%)。对于散发性病例,SRS 后 6 个月时 LC 率为 100%,12-60 个月时为 80%(95%可信区间:52%,100%)(p=0.39)。没有发生不良的放射事件或死亡。

结论

SRS 在幕上 HBs 的临床过程中显示出有希望的作用。它可以被认为是手术切除的有效替代方法,甚至在适当选择的病例中可以作为一线治疗选择。

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