Mathieu P, Favrot M, Frappaz D, Chauvin F, Greffe J, Montegue A, Lacroix C, David L, Brunat-Mentigny M, Philip T
Service de biologie générale et de neurobiollgie, CHS du Vinatier, Bron-Lyon, France.
Ann Biol Clin (Paris). 1993;51(7-8):665-88.
Neuroblastoma is the commonest solid tumour in children under the age of 5 years (50% of cases before 2 years, 90% before 5) and the second cause of death after accidents. Approximately one child in 10,000 develops neuroblastoma by the age of 15 years. The situation in other European and North American countries is similar to that in France. As neuroblastoma is derived from the sympathetic nervous tissues, it is associated with the production of large amounts of catecholamines and their metabolites which are excreted in the urine. Less than 5% of cases do not produce catecholamines. Vanillylmandelic acid (VMA), homovanillic acid (HVA) and dopamine (DA) are the most useful chemical markers for the diagnosis and clinical control of neuroblastoma. They are generally measured using the reliable and sensitive high pressure liquid chromatography (HPLC). Survival is related to stage (the Evan's staging protocol has been superseded by the INNS staging), and age at diagnosis. There is almost 100% survival for stages I and IIa before the age of 12 months, and less than 20% for stage IV when diagnosed after 2 years of age. Multiple copies of the N-myc oncogene, deletions of chromosome 1p, and diploidy in tumour cells are associated with poor prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)
神经母细胞瘤是5岁以下儿童最常见的实体瘤(50%的病例在2岁前发病,90%在5岁前发病),是仅次于意外事故的第二大致死原因。每10000名儿童中约有1名在15岁前会患上神经母细胞瘤。其他欧洲和北美国家的情况与法国类似。由于神经母细胞瘤起源于交感神经组织,它与大量儿茶酚胺及其代谢产物的产生有关,这些物质会通过尿液排出。不到5%的病例不产生儿茶酚胺。香草扁桃酸(VMA)、高香草酸(HVA)和多巴胺(DA)是神经母细胞瘤诊断和临床监测最有用的化学标志物。它们通常采用可靠且灵敏的高压液相色谱法(HPLC)进行检测。生存率与分期(埃文斯分期方案已被国际神经母细胞瘤分期系统取代)以及诊断时的年龄有关。12个月前诊断为I期和IIa期的患儿生存率几乎为100%,2岁后诊断为IV期的患儿生存率则不到20%。N - myc癌基因的多拷贝、1号染色体短臂缺失以及肿瘤细胞的二倍体与预后不良有关。(摘要截选至250词)