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髓母细胞瘤患儿肿瘤细胞倍性与生存的关系。

Relation of tumor-cell ploidy to survival in children with medulloblastoma.

作者信息

Gajjar A J, Heideman R L, Douglass E C, Kun L E, Kovnar E H, Sanford R A, Fairclough D L, Ayers D, Look A T

机构信息

Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105.

出版信息

J Clin Oncol. 1993 Nov;11(11):2211-7. doi: 10.1200/JCO.1993.11.11.2211.

Abstract

PURPOSE

To assess the value of tumor-cell ploidy as a predictor of survival in medulloblastoma.

PATIENTS AND METHODS

Ploidy determinations were based on the flow-cytometric analysis of cellular DNA content in fresh tumor specimens taken from 34 consecutively treated children with newly diagnosed medulloblastoma. Patients were assigned a high or low risk of failure depending on tumor size and invasiveness, and the presence or absence of metastatic disease. Treatment consisted of radiotherapy, with or without chemotherapy, according to institutional or cooperative group protocols.

RESULTS

Univariate analysis of candidate prognostic factors showed that only tumor-cell ploidy and clinical risk group had a statistically significant influence on survival. Patients with hyperdiploid stem lines (n = 9) had significantly longer survival times (P = .04) than did those with diploid lines (n = 20). The estimated 5-year survival probabilities (+/- SE) for these two subgroups were 89% +/- 11% and 48% +/- 13%, respectively. Although clinical risk status (high v low) showed essentially the same predictive strength as ploidy, the two features identified largely nonoverlapping subgroups. Thus, within the clinical high-risk group, it was possible to distinguish hyperdiploid patients whose 5-year survival rate (83% +/- 15%) was comparable to that of patients with localized, low-risk tumors.

CONCLUSION

This prospective study indicates that both ploidy and clinical risk group are important prognostic factors in medulloblastoma. Their combined use at diagnosis would distinguish patients who require more aggressive therapeutic intervention (diploid, clinical high-risk group) from those who could be expected to benefit most from standard treatment.

摘要

目的

评估肿瘤细胞倍性作为髓母细胞瘤生存预测指标的价值。

患者与方法

倍性测定基于对34例新诊断为髓母细胞瘤且接受连续治疗的儿童新鲜肿瘤标本中细胞DNA含量的流式细胞术分析。根据肿瘤大小、侵袭性以及有无转移性疾病,将患者分为高失败风险或低失败风险组。治疗依据机构或协作组方案,采用放疗,联合或不联合化疗。

结果

对候选预后因素的单因素分析显示,仅肿瘤细胞倍性和临床风险组对生存有统计学显著影响。超二倍体干细胞系患者(n = 9)的生存时间显著长于二倍体细胞系患者(n = 20)(P = 0.04)。这两个亚组的估计5年生存概率(±标准误)分别为89% ± 11%和48% ± 13%。尽管临床风险状态(高风险与低风险)显示出与倍性基本相同的预测强度,但这两个特征所确定的亚组在很大程度上不重叠。因此,在临床高风险组中,可以区分出5年生存率(83% ± 15%)与局限性低风险肿瘤患者相当的超二倍体患者。

结论

这项前瞻性研究表明,倍性和临床风险组都是髓母细胞瘤重要的预后因素。在诊断时联合使用这两个因素,可将需要更积极治疗干预的患者(二倍体、临床高风险组)与预期能从标准治疗中获益最大的患者区分开来。

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