Oppedal B R, Oien O, Jahnsen T, Brandtzaeg P
Laboratory for Immunohistochemistry and Immunopathology, University of Oslo, National Hospital, Norway.
J Clin Pathol. 1989 Nov;42(11):1148-52. doi: 10.1136/jcp.42.11.1148.
The association between tumour N-myc amplification, DNA ploidy, and various prognostic factors (patient age, tumour stage at diagnosis, primary site and histopathological differentiation) was studied in 18 children who had neuroblastoma, ganglioneuroblastoma, or ganglioneuroma. Amplification of genomic N-myc was observed in six patients who had been treated with chemotherapy before surgery (one with stage III and five with stage IV). All these tumours were located in the adrenal or upper retroperitoneum; five were neuroblastomas and one was a ganglioneuroblastoma. Three of them were aneuploid and three diploid. The degree of N-myc patients with tumour N-amplification varied from 20 to 1500 copies without relation to ploidy. All patients with tumour N-myc amplification died of their tumour. Amplification was always associated with poor prognosis, independent of tumour cell ploidy. Four patients without such amplification also died: three had diploid tumours, the fourth was aneuploid. It is suggested that genomic N-myc amplification takes place mainly in adrenal and retroperitoneal neuroblastomas and can be a feature of tumours with and without histological signs of differentiation. The precise role of N-myc in the pathogenesis of neuroblastoma remains unclear.
对18例患有神经母细胞瘤、神经节神经母细胞瘤或神经节神经瘤的儿童，研究了肿瘤N - myc扩增、DNA倍性与各种预后因素（患者年龄、诊断时的肿瘤分期、原发部位和组织病理学分化）之间的关联。在6例术前接受过化疗的患者中观察到基因组N - myc扩增（1例Ⅲ期，5例Ⅳ期）。所有这些肿瘤均位于肾上腺或上腹膜后；5例为神经母细胞瘤，1例为神经节神经母细胞瘤。其中3例为非整倍体，3例为二倍体。肿瘤N - myc扩增患者的N - myc拷贝数在20至1500之间，与倍性无关。所有肿瘤N - myc扩增患者均死于肿瘤。扩增总是与预后不良相关，与肿瘤细胞倍性无关。4例无此类扩增的患者也死亡：3例为二倍体肿瘤，第4例为非整倍体。提示基因组N - myc扩增主要发生在肾上腺和腹膜后神经母细胞瘤中，并且可以是有或无组织学分化迹象的肿瘤的一个特征。N - myc在神经母细胞瘤发病机制中的确切作用仍不清楚。
