Laug W E, Siegel S E, Shaw K N, Landing B, Baptista J, Gutenstein M
Pediatrics. 1978 Jul;62(1):77-83.
Initial urinary catecholamine metabolite and amino acid excretion patterns were examined in 54 children with neuroblastoma. The relationships between prognosis and age at diagnosis, stage of disease, primary site, and histologic grade of tumor were similar in this population to those found in previous studies, but only age and stage were found to be independent prognostic variables. Prognosis in disseminated disease was found to correlate directly with the urinary vanilmandelic acid (VMA)/homovanillic acid (HVA) ratio but not with the absolute levels of HVA. The presence of the dopa metabolite, vanillactic acid, as well as increased amounts of cystathionine and/or low levels of VMA indicated poor prognosis. These results are consistent with the hypothesis that biochemically primitive neuroblastomas deficient in dopamine beta-hydroxylase are move virulent than their mature analogues which produce epinephrine, norepinephrine, and their metabolites.
对54例神经母细胞瘤患儿的初始尿儿茶酚胺代谢产物和氨基酸排泄模式进行了检查。该人群中,诊断时的年龄、疾病分期、原发部位和肿瘤组织学分级与预后的关系与先前研究中发现的相似,但仅年龄和分期被发现是独立的预后变量。发现播散性疾病的预后与尿香草扁桃酸(VMA)/高香草酸(HVA)比值直接相关,而与HVA的绝对水平无关。多巴代谢产物香草乳酸的存在,以及胱硫醚含量增加和/或VMA水平降低表明预后不良。这些结果与以下假设一致,即缺乏多巴胺β-羟化酶的生化原始神经母细胞瘤比产生肾上腺素、去甲肾上腺素及其代谢产物的成熟类似物更具侵袭性。