[Tumor cytogenetics and prognosis in neuroblastoma].
[Tumor cytogenetics and prognosis in neuroblastoma].
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[神经母细胞瘤的肿瘤细胞遗传学和预后]。
DOI：
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发表时间：
1989
期刊：
影响因子：
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通讯作者：
F. Lampert
中科院分区：
文献类型：
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作者：
H. Christiansen;F. Lampert
对40例不同临床分期的神经母细胞瘤患儿，在发病（30例）或复发（10例）进行肿瘤组织短期培养或骨髓抽吸后测定肿瘤核型。10例I、II和IV期肿瘤均未出现染色体1p畸变，而在30例III和IV期肿瘤中，有25例（=83%）出现了染色体1p畸变。肿瘤dna中原癌基因N-myc扩增在I期、II期和IV期未检测到，但在53%的III期和IV期肿瘤中存在。基因扩增的细胞遗传学现象，如双分钟（DMs）和均匀染色区（HSRs）与N-myc扩增相关。大约50%的III期和IV期肿瘤的染色体数目在近二倍体范围内，而预后有利的肿瘤的特征是染色体数目主要在三倍体范围内的超倍体。Kaplan-Meier生命表分析显示，在肿瘤细胞中1号染色体形态正常的患者中，存活率约为80%，而在没有N-myc癌基因扩增的情况下，存活率约为60%，如果检测到非整倍体，存活率约为50%。因此，我们认为，肿瘤核型中染色体1p畸变的存在与否是神经母细胞瘤患儿预后最敏感的鉴别指标。
In 40 children with neuroblastoma of different clinical stages the tumorkaryotype was determined at onset (n = 30) or at relapse (n = 10) of disease after short-term culture of tumor tissues or bone marrow aspirates. None of the 10 stage I, II, and IVs tumors revealed a chromosome 1p aberration, in contrast to stage III and IV tumors where this abnormality was encountered in 25 (=83%) of 30 patients. Amplification of the proto-oncogene N-myc in the tumor-DNA could not be detected in stage I, II and IVs, was however present in 53% of stage III and IV tumors. Cytogenetic phenomena of gene amplification such as Double minutes (DMs) and Homogeneously Staining Regions (HSRs) correlated with N-myc amplification. About 50% of stage III and IV tumors had chromosome numbers in the neardiploid range whereas prognostically favourable tumors were characterized by hyperploid chromosomal numbers mainly in the triploid range. Life-table analysis according to Kaplan-Meier showed a probability of surviving in about 80% of patients with a normal morphology of chromosome 1 in their tumor cells, compared to about 60% in the absence of N-myc oncogene amplification and of about 50%, if aneuploidy is detected. Thus, we think, the presence or absence of chromosome 1p aberration in the tumorkaryotype is the most sensitive discriminator for outcome in children with neuroblastoma.