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后颅窝巨大实性血管母细胞瘤的治疗策略:13 例连续病例的回顾性分析。

Treatment Strategy for Giant Solid Hemangioblastomas in the Posterior Fossa: A Retrospective Review of 13 Consecutive Cases.

机构信息

Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

出版信息

World Neurosurg. 2022 Feb;158:e214-e224. doi: 10.1016/j.wneu.2021.10.169. Epub 2021 Oct 30.

Abstract

OBJECTIVE

To analyze radiologic characteristics, treatment strategy, and treatment outcomes of posterior fossa giant solid hemangioblastomas (GSHBs; ≥4 cm in maximum diameter).

METHODS

We performed a retrospective study of 13 consecutive patients (9 male and 4 female patients; mean age, 57.5 ± 13.3 years; range, 24-76 years) with GSHB who underwent surgical resection at a single institution between 2002 and 2018. The median follow-up was 33 months (range, 12-120 months). For each patient, neuroimaging findings, operative record, and treatment outcome were reviewed.

RESULTS

Seven tumors (53.8%) were located within cerebellar hemispheres, 4 (30.8%) in proximity to the brainstem, 1 (7.7%) within the cerebellar vermis, and 1 (7.7%) in the cerebellopontine angle. The mean maximum diameter was 4.8 ± 0.8 cm (range, 4.0-6.7 cm). Gross total resection was achieved in 11 patients (84.6%), and near-total resection in 2 patients (15.4%). Surgical complications occurred in 5 patients (38.5%); persistent neurologic deficits occurred in 2 patients (15.4%). Estimated progression-free survival after surgery was 92.3%, 80.8%, and 80.8% at 1, 5, and 10 years, respectively, whereas the estimated 1-year, 5-year, and 10-year overall survival was 100%, 90%, and 90%, respectively.

CONCLUSIONS

GSHBs are surgically challenging. The current study shows that favorable outcome can be achieved for GSHBs in the cerebellar hemispheres and vermis. For those involving the brainstem, planned near-total resection or subtotal resection in a piecemeal fashion can be attempted if en bloc resection is judged to be infeasible, and further intervention can be considered as needed.

摘要

目的

分析后颅窝巨大实性血管母细胞瘤(GSHB;最大直径≥4cm)的影像学特征、治疗策略和治疗结果。

方法

我们对 2002 年至 2018 年间在一家单机构接受手术切除的 13 例连续 GSHB 患者(9 例男性和 4 例女性患者;平均年龄 57.5±13.3 岁;范围 24-76 岁)进行了回顾性研究。每位患者的神经影像学表现、手术记录和治疗结果均进行了回顾。

结果

7 例肿瘤(53.8%)位于小脑半球内,4 例(30.8%)靠近脑干,1 例(7.7%)位于小脑蚓部,1 例(7.7%)位于桥小脑角。最大直径平均值为 4.8±0.8cm(范围 4.0-6.7cm)。11 例患者(84.6%)实现了全切除,2 例患者(15.4%)实现了近全切除。5 例患者(38.5%)发生手术并发症;2 例患者(15.4%)出现持续性神经功能缺损。术后无进展生存估计分别为 1 年、5 年和 10 年时的 92.3%、80.8%和 80.8%,而估计的 1 年、5 年和 10 年总生存率分别为 100%、90%和 90%。

结论

GSHB 手术难度大。本研究表明,对于小脑半球和蚓部的 GSHB,可获得良好的结果。对于涉及脑干的 GSHB,如果认为整块切除不可行,可以尝试计划行近全切除或分块次全切除,如有需要可进一步干预。

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