Disseminated neuroblastomas under 1 year of age: Cell biology and prognosis
Disseminated neuroblastomas under 1 year of age: Cell biology and prognosis
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DOI：
10.1023/a:1005778607661
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发表时间：
1997-01-01
影响因子：
3.9
通讯作者：
Berthold, F
中科院分区：
文献类型：
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作者：
Lampert, F;Christiansen, H;Berthold, F
203例不同临床阶段的神经母细胞瘤婴儿的肿瘤标本-在德国儿科肿瘤学会的连续多中心临床试验中注册-可以检查N-myc扩增，染色体l-倍性和结构，CD 44标准。表达其中87例（= 43%）为非局限性、播散性神经母细胞瘤，主要累及交感神经组织、淋巴结、肝脏、皮肤、骨髓和骨骼（4（s）组46例，真4组41例）如果忽略4期和4（s）期之间的临床分型，然后这些婴儿中的17个（= 20%）具有N-myc扩增（4-64个拷贝），其中16个已经死亡。9例真性4期患者中7例有染色体Ip畸变（5例N-myc扩增），死亡者中2例CD 44阴性。通过ELISA测量，在4期婴儿（n = 6）中发现最高（显著不同）Kruskal-Wallis平均等级值（147.8），而在4期患者（n = 65）中发现低平均等级值71.9。1-3期患儿（n = 42）的CD 44值为99.8- 88.6。因此，表现为非二倍体、染色体Ip结构正常、无N-myc扩增和高CD 44标准的播散性神经母细胞瘤的婴儿。在肿瘤组织中的表达，以及高的CD 44标准。另一方面，发现肿瘤细胞中的N-myc扩增是具有肿瘤进展的4期神经母细胞瘤患者（n = 6）的特征。因此，4（s）神经母细胞瘤-N-myc扩增肿瘤患者应积极治疗真正的4期肿瘤患者！
Tumor specimens of 203 infants with neuroblastomas of different clinical stages - registered in successive multicenter clinical trials of the German Society of Pediatric Oncology - could be examined for N-myc amplification, chromosome l-ploidy and - structure, CD44 std. expression (in tumor tissue, and also in patient's sera).Eightyseven (= 43%) of these infants had a non-localized, disseminated neuroblastoma, mainly involving sympathetic nerve tissue,lymphnodes, liver, skin, bone marrow and bones (46 patients were classified into the 4(s) group, 41 patients in the true 4 group).If the clinical classification between stage 4 and stage 4(s) was neglected, then 17 of these infants (= 20%) had N-myc amplification (4-64 copies) with 16 already dead. Seven of 9 examined patients with true stage 4 had chromosome Ip aberrations (with N-myc amplification in 5), and among the dead there were 2 with CD44 negative expression.In another series, serum CD44 std. was measured by ELISA, and the highest (significantly different) Kruskal-Wallis mean rank values (147.8) were found in infants (n = 6) with stage 4(s) compared to the low mean rank-value of 71.9 in patients with stage 4 (n = 65). Stage 1-3 patients (n = 42) had values of 99.8-88.6.Thus, infants with disseminated neuroblastomas, showing non-diploidy, normal chromosome Ip structure, non-N-myc amplification and high CD44 std. expression in tumor tissue, and also high CD44 std. values in serum, will have the highest chance of survival due to tumor-non-progression.On the other hand, N-myc amplification in the tumor cells was found to be characteristic for stage 4(s) neuroblastoma patients with tumor progression (n = 6). Therefore, 4(s) neuroblastoma-patients with N-myc amplified tumors should be aggressively treated like true stage 4 tumor patients!