Woods W G, Lemieux B, Tuchman M
Department of Pediatrics, University of Minnesota, Minneapolis 55455.
Pediatrics. 1992 Jan;89(1):114-8.
Data are presented suggesting that neuroblastoma represents at least two distinct clinical-biologic entities. One, favorable neuroblastoma, is associated with young age and early stage at diagnosis, triploid karyotypes, no 1p abnormalities or N-myc gene amplification, more mature catecholamine synthesis and excretion, and excellent clinical outcome despite no or minimal therapy. The other, unfavorable neuroblastoma, is associated with older age and advanced stage, pseudodiploid karyotypes and 1p deletions, N-myc oncogene amplification, less mature catecholamine synthesis and excretion, and poor outcome despite aggressive multimodality therapy including bone marrow transplantation. Favorable neuroblastomas rarely, if ever, evolve into unfavorable disease. Unresolved issues regarding this hypothesis and implications for the efficacy of preclinical detection through mass screening are discussed.
所呈现的数据表明，神经母细胞瘤至少代表两种不同的临床生物学实体。一种是预后良好的神经母细胞瘤，与诊断时年龄小、分期早、三倍体核型、无1p异常或N - myc基因扩增、儿茶酚胺合成及排泄更成熟有关，且即便未接受治疗或仅接受极少治疗，临床预后也极佳。另一种是预后不良的神经母细胞瘤，与年龄较大、分期较晚、假二倍体核型及1p缺失、N - myc癌基因扩增、儿茶酚胺合成及排泄不太成熟有关，且尽管接受了包括骨髓移植在内的积极多模式治疗，预后仍很差。预后良好的神经母细胞瘤极少（如果有的话）演变为预后不良的疾病。本文讨论了关于这一假说的未解决问题以及通过大规模筛查进行临床前检测的有效性的影响。
