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性腺外恶性生殖细胞肿瘤患儿的预后因素:一项儿科多中心研究

Prognostic factors in children with extragonadal malignant germ cell tumors: a pediatric intergroup study.

作者信息

Marina Neyssa, London Wendy B, Frazier A Lindsay, Lauer Stephen, Rescorla Frederick, Cushing Barbara, Malogolowkin Marcio H, Castleberry Robert P, Womer Richard B, Olson Thomas

机构信息

Stanford University Medical Center, Stanford, CA 94304-1812, USA.

出版信息

J Clin Oncol. 2006 Jun 1;24(16):2544-8. doi: 10.1200/JCO.2005.04.1251.

DOI:10.1200/JCO.2005.04.1251
PMID:16735707
Abstract

PURPOSE

To investigate prognostic factors for pediatric extragonadal malignant germ cell tumors (PEMGCT).

MATERIALS AND METHODS

Between 1990 and 1996, patients with stage I through IV PEMGCT were eligible for a trial of cisplatin dose intensity. We retrospectively investigated prognostic factors for PEMGCT, including age, stage, primary site, treatment, and elevated alfa fetoprotein by univariate and multivariate analysis.

RESULTS

The 165 patients had a median age of 1.9 years (range, 3 days to 18.5 years); 109 were female; and 99 had alfa fetoprotein > or = 10,000. There were 30 stage I/II, 61 stage III, and 74 stage IV tumors; primary sites included 88 sacrococcygeal, 39 thoracic, and 38 others. The 5-year overall survival (OS) and event-free survival (EFS) rates with standard deviations were 83.4% +/- 3.7% and 79.0% +/- 4.1%, respectively. Univariate analysis identified age > or = 12 years as a highly significant prognostic factor for EFS (5-year EFS, 48.9% +/- 15.6% v 84.1% +/- 3.9%; P < .0001) and for OS (5-year OS, 53.7% +/- 14.9% v 88.5% +/- 3.4%; P < .0001), whereas treatment was of borderline significance (P = .0777). Multivariate Cox proportional hazards regression identified only age > or = 12 years as a significant prognostic factor for EFS (P = .0002). In multivariate Cox regression for OS, the combination of age and primary site was highly significant (P < .0001). Patients > or = 12 years of age with thoracic tumors had six times the risk of death compared with patients younger than 12 years with other primaries.

CONCLUSION

Age is the most predictive factor of EFS in PEMGCT. There is a significant interaction between age and primary site, suggesting that patients > or = 12 years of age with thoracic tumors are a biologically distinct group.

摘要

目的

探讨小儿性腺外恶性生殖细胞肿瘤(PEMGCT)的预后因素。

材料与方法

1990年至1996年间,I期至IV期PEMGCT患者 eligible for a trial of cisplatin dose intensity(这个表述有误,可能是“符合顺铂剂量强度试验条件”之类的意思)。我们通过单因素和多因素分析回顾性研究了PEMGCT的预后因素,包括年龄、分期、原发部位、治疗以及甲胎蛋白升高情况。

结果

165例患者的中位年龄为1.9岁(范围3天至18.5岁);109例为女性;99例甲胎蛋白≥10,000。有30例I/II期、61例III期和74例IV期肿瘤;原发部位包括88例骶尾部、3例胸部和38例其他部位。5年总生存率(OS)和无事件生存率(EFS)及其标准差分别为83.4%±3.7%和79.0%±4.1%。单因素分析确定年龄≥12岁是EFS(5年EFS,48.9%±15.6%对84.1%±3.9%;P<.0001)和OS(5年OS,53.7%±14.9%对88.5%±3.4%;P<.0001)的高度显著预后因素,而治疗具有临界显著性(P = 0.0777)。多因素Cox比例风险回归仅确定年龄≥12岁是EFS的显著预后因素(P = 0.0002)。在OS的多因素Cox回归中,年龄和原发部位的组合具有高度显著性(P<.0001)。年龄≥12岁的胸部肿瘤患者的死亡风险是年龄小于12岁的其他原发肿瘤患者的6倍。

结论

年龄是PEMGCT中EFS的最具预测性的因素。年龄和原发部位之间存在显著相互作用,表明年龄≥12岁的胸部肿瘤患者是一个生物学上不同的群体。

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