Hiyama E, Yokoyama T, Ichikawa T, Hiyama K, Kobayashi M, Tanaka Y, Ueda K, Tanaka Y, Yano H
Department of General Medicine, Hiroshima University School of Medicine, Japan.
Cancer. 1994 Sep 15;74(6):1821-6. doi: 10.1002/1097-0142(19940915)74:6<1821::aid-cncr2820740627>3.0.co;2-a.
Most patients with neuroblastoma who present with opsomyoclonus have a good prognosis. Neuroblastomas from such patients have been reported to contain a single copy of the N-myc gene. The authors describe three cases of patients with advanced neuroblastoma with opsomyoclonus, which had poor outcomes despite multimodal therapy.
Amplification and expression of the N-myc gene were examined in these three primary tumors using Southern and Northern blot analyses. Then, flow cytometric analysis of the cellular DNA contents of these tumors was performed.
N-myc amplification was observed in two tumors and N-myc RNA overexpression was observed in all three. Analysis of the cellular DNA contents of the tissue specimens revealed hyperdiploidy in all three tumors; one had a triploid index and the other two had hypotetraploid indexes.
These findings suggest that a poor outcome for patients with opsomyoclonus may be associated with N-myc gene activation (amplification and/or overexpression) and that a hyperdiploid tumor is not always associated with a good prognosis.
大多数出现眼肌阵挛的神经母细胞瘤患者预后良好。据报道，此类患者的神经母细胞瘤含有单拷贝的N - myc基因。作者描述了三例患有进展期神经母细胞瘤并伴有眼肌阵挛的患者，尽管接受了多模式治疗，但其预后较差。
使用Southern和Northern印迹分析检测这三个原发性肿瘤中N - myc基因的扩增和表达。然后，对这些肿瘤的细胞DNA含量进行流式细胞术分析。
在两个肿瘤中观察到N - myc扩增，在所有三个肿瘤中均观察到N - myc RNA过表达。对组织标本的细胞DNA含量分析显示，所有三个肿瘤均为超二倍体；一个具有三倍体指数，另外两个具有亚四倍体指数。
这些发现表明，眼肌阵挛患者预后不良可能与N - myc基因激活（扩增和/或过表达）有关，并且超二倍体肿瘤并不总是与良好的预后相关。
