Fong C T, White P S, Peterson K, Sapienza C, Cavenee W K, Kern S E, Vogelstein B, Cantor A B, Look A T, Brodeur G M
Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110.
Cancer Res. 1992 Apr 1;52(7):1780-5.
Neuroblastomas have been characterized genetically by N-myc amplification and by deletions or loss of heterozygosity (LOH) for the short arm of chromosome 1. However, recent studies have suggested deletion or allelic loss involving at least three other chromosome arms, 11q, 14q, and 17p. Therefore, we undertook an analysis of allelic loss for these respective chromosomal arms to determine the frequency and pattern of LOH as well as the correlation of these findings with other biological and clinical variables. A group of 24 pairs of normal and tumor DNAs was chosen that were representative of patients of different ages and stages. A substantial frequency of LOH (greater than or equal to 20%) was found only for 1p and 14q, whereas LOH for the other chromosome arms occurred in less than or equal to 5% of cases. On the basis of these results, we extended the analysis to a total of 59 neuroblastomas, and we found 1p LOH in 15 of the 59 cases (25%) and 14q LOH in 10 of 43 informative cases (23%). N-myc amplification was found in 15 of the 59 cases (25%). This analysis confirmed that 1p LOH and 14q LOH occurred almost exclusively in patients with advanced stages of disease. Furthermore, LOH for 1p and 14q usually occurred independent of each other, and 1p LOH frequently was associated with N-myc amplification, whereas 14q LOH was not. Thus, our results demonstrate that neuroblastomas are complex genetically and that there are at least two distinct loci for putative suppressor genes that are deleted independently in this tumor, both of which are associated with advanced stages of disease.
神经母细胞瘤已通过N - myc基因扩增以及1号染色体短臂的缺失或杂合性丢失(LOH)进行基因特征分析。然而,最近的研究表明,至少还有其他三条染色体臂11q、14q和17p存在缺失或等位基因丢失。因此,我们对这些染色体臂进行等位基因丢失分析,以确定LOH的频率和模式,以及这些发现与其他生物学和临床变量的相关性。选择了一组24对正常和肿瘤DNA,它们代表了不同年龄和阶段的患者。仅在1p和14q上发现了较高频率的LOH(大于或等于20%),而其他染色体臂的LOH发生率小于或等于5%。基于这些结果,我们将分析扩展至总共59例神经母细胞瘤,发现在59例中有15例(25%)存在1p LOH,在43例信息充分的病例中有10例(23%)存在14q LOH。在59例中有15例(25%)发现N - myc基因扩增。该分析证实,1p LOH和14q LOH几乎仅发生在疾病晚期患者中。此外,1p和14q的LOH通常相互独立发生,1p LOH常与N - myc基因扩增相关,而14q LOH则不然。因此,我们的结果表明神经母细胞瘤在基因上是复杂的,并且在该肿瘤中至少有两个不同的假定抑癌基因位点会独立缺失,这两个位点均与疾病晚期相关。