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台湾地区小儿基底节生殖细胞瘤治疗结果的单中心研究。

A single-center study of treatment outcomes of pediatric basal ganglia germinoma in Taiwan.

作者信息

Kang Yu-Mei, Lin Shih-Chieh, Lee Yi-Yen, Chang Feng-Chi, Liang Muh-Lii, Chen Hsin-Hung, Wong Tai-Tong, Chen Yi-Wei

机构信息

Faculty of Medicine, National Yang-Ming University, Taipei, Taiwan.

Division of Radiation Oncology, Department of Oncology, Taipei Veterans General Hospital, Taipei, Taiwan.

出版信息

Childs Nerv Syst. 2020 Aug;36(8):1745-1753. doi: 10.1007/s00381-020-04543-4. Epub 2020 Feb 24.

Abstract

PURPOSE

A basal ganglia (BG) germinoma is a rare tumor, and the optimal treatment remains unknown. We evaluated the clinical outcomes of treatment of BG germinoma in pediatric patients in Taiwan.

METHODS

We retrospectively reviewed the medical records of 34 children with BG germinoma who were treated with radiotherapy (RT) at Taipei Veterans General Hospital between 1989 and 2016. The median follow-up time is 8.3 years (1.8-25.2 years). Survival was analyzed using the Kaplan-Meier estimate. Univariate Cox proportional-hazards models were used to identify the potential risk factors.

RESULTS

Only four patients (11.8%) experienced recurrence and all successfully underwent salvage therapy. One patient (2.97%) died due to suspected radiotherapy (RT)-related sarcoma in the scalp. The 2-, 3-, and 5-year DFS rates were 91.2%, 88.2%, and 79.4%, respectively; the 2-, 3-, and 5-year OS rates were 97.1%, 94.1%, and 82.4%, respectively. Focal RT showed low DFS in the Kaplan-Meier survival curves (P = .028) compared with non-focal RT (whole ventricle, whole brain, or cranial spinal area). In the univariate Cox proportional-hazards model, there was a significant difference in DFS between focal and non-focal RT (P = .03). There is no difference in DFS and OS between BG germinoma patients and non-BG germinoma patients.

CONCLUSIONS

We found an excellent DFS and OS in pediatric patients with BG germinoma treated with RT. Whole ventricle irradiation is recommended for good tumor control and low treatment-related toxicity. BG germinoma patients showed similar treatment results as germinoma patients in other common sites.

摘要

目的

基底节区(BG)生殖细胞瘤是一种罕见肿瘤,最佳治疗方案仍不明确。我们评估了台湾地区儿童BG生殖细胞瘤的治疗临床结局。

方法

我们回顾性分析了1989年至2016年间在台北荣民总医院接受放射治疗(RT)的34例儿童BG生殖细胞瘤患者的病历。中位随访时间为8.3年(1.8 - 25.2年)。采用Kaplan-Meier法估计生存率。使用单因素Cox比例风险模型确定潜在危险因素。

结果

仅4例患者(11.8%)复发,均成功接受挽救治疗。1例患者(2.97%)因疑似放疗相关的头皮肉瘤死亡。2年、3年和5年无病生存率(DFS)分别为91.2%、88.2%和79.4%;2年、3年和5年总生存率(OS)分别为97.1%、94.1%和82.4%。在Kaplan-Meier生存曲线中,与非局部放疗(全脑室、全脑或全脊髓区)相比,局部放疗的DFS较低(P = 0.028)。在单因素Cox比例风险模型中,局部放疗与非局部放疗的DFS有显著差异(P = 0.03)。BG生殖细胞瘤患者与非BG生殖细胞瘤患者的DFS和OS无差异。

结论

我们发现接受放疗的儿童BG生殖细胞瘤患者的DFS和OS良好。建议采用全脑室照射以实现良好的肿瘤控制并降低治疗相关毒性。BG生殖细胞瘤患者的治疗结果与其他常见部位的生殖细胞瘤患者相似。

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