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儿童及青年期颅内生殖细胞瘤的长期预后及晚期效应

Long-term outcomes and late effects for childhood and young adulthood intracranial germinomas.

作者信息

Acharya Sahaja, DeWees Todd, Shinohara Eric T, Perkins Stephanie M

机构信息

Department of Radiation Oncology, Washington University School of Medicine, St. Louis, Missouri (S.A., T.D., S.M.P.); Department of Radiation Oncology, Vanderbilt University School of Medicine, Nashville, Tennessee (E.T.S.).

出版信息

Neuro Oncol. 2015 May;17(5):741-6. doi: 10.1093/neuonc/nou311. Epub 2014 Nov 23.

Abstract

BACKGROUND

Pediatric and young adult central nervous system (CNS) germinomas have favorable cure rates. However, long-term follow-up data are limited because of the rarity of this tumor. We report the long-term overall survival (OS) and causes of late mortality for these patients.

METHODS

Data between 1973 and 2005 from the Surveillance, Epidemiology, and End Results (SEER) database were analyzed. Kaplan Meier survival analysis was performed on 5-year survivors of childhood CNS germinomatous germ cell tumors (GGCTs) and nongerminomatous germ cell tumors (NGGCTs). Standardized mortality ratios (SMRs) were calculated using US population data to compare observed versus expected all-cause death and death from stroke. Cumulative incidence was calculated using a competing risk model.

RESULTS

Four hundred five GGCTs and 94 NGGCTs cases were eligible. OS at 20 and 30 years for GGCTs was 84.1% and 61.9%, respectively, and was 86.7% for NGGCTs at both time points. Five-year survivors of GGCTs and NGGCTs experienced a 10-fold increase in mortality risk compared with their peers (SMR, 10.41; 95% confidence interval [CI], 7.71-13.76 vs SMR, 10.39;95% CI, 4.83-19.73, respectively). Five-year survivors GGCTs also experienced a nearly 59-fold increase in risk of death from stroke (SMR, 58.93; 95% CI, 18.72-142.10). At 25 years, the cumulative incidence of death due to cancer and subsequent malignancy was 16% and 6.0%, respectively.

CONCLUSION

Although CNS germinomas have favorable cure rates, late recurrences, subsequent malignancies, and stroke significantly affect long-term survival. Close attention to long-term follow-up with assessment of stroke risk factors is recommended.

摘要

背景

儿童及青年中枢神经系统(CNS)生殖细胞瘤的治愈率较高。然而,由于该肿瘤罕见,长期随访数据有限。我们报告了这些患者的长期总生存率(OS)及晚期死亡原因。

方法

分析了监测、流行病学和最终结果(SEER)数据库1973年至2005年的数据。对儿童CNS生殖细胞性生殖细胞瘤(GGCT)和非生殖细胞性生殖细胞瘤(NGGCT)的5年幸存者进行了Kaplan Meier生存分析。使用美国人口数据计算标准化死亡比(SMR),以比较观察到的全因死亡和中风死亡与预期情况。使用竞争风险模型计算累积发病率。

结果

405例GGCT和94例NGGCT病例符合条件。GGCT在20年和30年时的OS分别为84.1%和61.9%,NGGCT在这两个时间点的OS均为86.7%。GGCT和NGGCT的5年幸存者的死亡风险比同龄人增加了10倍(SMR分别为10.41;95%置信区间[CI],7.71 - 13.76和SMR为10.39;95% CI,4.83 - 19.73)。GGCT的5年幸存者中风死亡风险也增加了近59倍(SMR,58.93;95% CI,18.72 - 142.10)。在25年时,因癌症和后续恶性肿瘤导致的死亡累积发病率分别为16%和6.0%。

结论

尽管CNS生殖细胞瘤治愈率较高,但晚期复发、后续恶性肿瘤和中风显著影响长期生存。建议密切关注长期随访并评估中风风险因素。

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