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儿童丘脑和丘脑底节低级别胶质瘤的生存和功能结局。

Survival and functional outcomes in paediatric thalamic and thalamopeduncular low grade gliomas.

机构信息

Department of Neurosurgery, Second Faculty of Medicine, Charles University and Motol University Hospital, V Úvalu 84, Prague, Czech Republic.

Department of Paediatric Haematology and Oncology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.

出版信息

Acta Neurochir (Wien). 2022 Jun;164(6):1459-1472. doi: 10.1007/s00701-021-05106-5. Epub 2022 Jan 19.

Abstract

BACKGROUND

Childhood thalamopeduncular gliomas arise at the interface of the thalamus and cerebral peduncle. The optimal treatment is total resection but not at the cost of neurological function. We present long-term clinical and oncological outcomes of maximal safe resection.

METHODS

Retrospective review of prospectively collected data: demography, symptomatology, imaging, extent of resection, surgical complications, histology, functional and oncological outcome.

RESULTS

During 16-year period (2005-2020), 21 patients were treated at our institution. These were 13 girls and 8 boys (mean age 7.6 years). Presentation included progressive hemiparesis in 9 patients, raised intracranial pressure in 9 patients and cerebellar symptomatology in 3 patients. The tumour was confined to the thalamus in 6 cases. Extent of resection was judged on postoperative imaging as total (6), near-total (6) and less extensive (9). Surgical complications included progression of baseline neurological status in 6 patients, and 5 of these gradually improved to preoperative status. All tumours were classified as low-grade gliomas. Disease progression was observed in 9 patients (median progression-free survival 7.3 years). At last follow-up (median 6.1 years), all patients were alive, median Lansky score of 90. Seven patients were without evidence of disease, 6 had stable disease, 7 stable following progression and 1 had progressive disease managed expectantly.

CONCLUSION

Paediatric patients with low-grade thalamopeduncular gliomas have excellent long-term functional and oncological outcomes when gross total resection is not achievable. Surgery should aim at total resection; however, neurological function should not be endangered due to excellent chance for long-term survival.

摘要

背景

儿童丘脑脑桥胶质瘤位于丘脑和大脑脚交界处。最佳治疗方法是完全切除,但不能以牺牲神经功能为代价。我们介绍最大限度安全切除的长期临床和肿瘤学结果。

方法

回顾性分析前瞻性收集的数据:人口统计学、症状、影像学、切除范围、手术并发症、组织学、功能和肿瘤学结果。

结果

在 16 年期间(2005-2020 年),我们机构治疗了 21 名患者。其中 13 名女孩和 8 名男孩(平均年龄 7.6 岁)。表现包括 9 例进行性偏瘫、9 例颅内压升高和 3 例小脑症状。肿瘤局限于丘脑 6 例。根据术后影像学判断切除范围为全切除(6 例)、近全切除(6 例)和非广泛切除(9 例)。手术并发症包括 6 例基线神经状态进展,其中 5 例逐渐恢复术前状态。所有肿瘤均分类为低级别胶质瘤。9 例患者观察到疾病进展(无进展生存期的中位数为 7.3 年)。末次随访(中位数 6.1 年)时,所有患者均存活,Lansky 评分中位数为 90。7 例无疾病证据,6 例疾病稳定,7 例进展后稳定,1 例进展性疾病期待治疗。

结论

当不能达到完全切除时,儿童低级别丘脑脑桥胶质瘤患者具有极好的长期功能和肿瘤学结果。手术应旨在完全切除;然而,由于长期生存的机会极好,不应因神经功能受损而危及手术。

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