Bernstein M L, Leclerc J M, Bunin G, Brisson L, Robison L, Shuster J, Byrne T, Gregory D, Hill G, Dougherty G
Department of Pediatrics, McGill University, Montreal Children's Hospital, Quebec, Canada.
J Clin Oncol. 1992 Feb;10(2):323-9. doi: 10.1200/JCO.1992.10.2.323.
The purpose of this study was twofold: (1) to provide a population-based estimate of neuroblastoma incidence, disease stage and age distribution, and survival and mortality rates in North America; and (2) to compare these figures in the province of Quebec at a time shortly before the institution of province-wide screening with those in a population-based control group, the Greater Delaware Valley (GDV) Pediatric Tumor Registry.
In Quebec, the four major pediatric teaching hospital records were searched for children with a diagnosis of neuroblastoma. Tumor board registry data and information supplied to the Division of Vital Statistics were also reviewed. Birth statistics were obtained from the population registry. The GDV Pediatric Tumor Registry is a population-based registry of pediatric cancer covering all of Delaware and parts of New Jersey, Pennsylvania, and Maryland. Age, stage of disease, and follow-up data were obtained through December 31, 1989, with Evans neuroblastoma staging data used for all comparisons.
One hundred thirty children with neuroblastoma were identified in Quebec and 165 in the GDV, in a combined population of 3,178,736 children. The annual incidence of neuroblastoma was 10.95/10(6) under the age of 15 years and 27.75/10(6) between the ages of 0 and 4 years. The annual mortality rate due to neuroblastoma was 4.89/10(6) and 9.10/10(6) for the age groups 0 to 14 and 0 to 4, respectively. The overall 10-year survival rate for the 295 cases of neuroblastoma was 55%. The 10-year survival rates for patients with Evans stage I-IV and IVS disease were 88%, 90%, 63%, 21%, and 81%. There was no significant difference observed in the incidence, mortality, or survival in the two populations.
These data represent the first large, population-based description of the clinical presentation and outcome of patients with neuroblastoma in North America, with no significant differences noted between Quebec patients and the GDV patients.
本研究有两个目的：（1）对北美神经母细胞瘤的发病率、疾病分期、年龄分布、生存率和死亡率进行基于人群的估计；（2）在魁北克省全省范围筛查实施前不久，将该省的数据与基于人群的对照组——大特拉华谷（GDV）儿科肿瘤登记处的数据进行比较。
在魁北克，检索了四家主要儿科教学医院中诊断为神经母细胞瘤的儿童记录。还审查了肿瘤委员会登记数据以及提供给生命统计部门的信息。出生统计数据来自人口登记处。GDV儿科肿瘤登记处是一个基于人群的儿科癌症登记处，涵盖了整个特拉华州以及新泽西州、宾夕法尼亚州和马里兰州的部分地区。年龄、疾病分期和随访数据截至1989年12月31日，所有比较均使用埃文斯神经母细胞瘤分期数据。
在魁北克省共识别出130例神经母细胞瘤患儿，在GDV识别出165例，研究人群共计3178736名儿童。神经母细胞瘤的年发病率在15岁以下儿童中为10.95/10⁶，在0至4岁儿童中为27.75/10⁶。神经母细胞瘤的年死亡率在0至14岁年龄组中为4.89/10⁶，在0至4岁年龄组中为9.10/10⁶。295例神经母细胞瘤患者的总体10年生存率为55%。埃文斯I - IV期和IVS期疾病患者的10年生存率分别为88%、90%、63%、21%和81%。在这两个人群的发病率、死亡率或生存率方面未观察到显著差异。
这些数据首次对北美神经母细胞瘤患者的临床表现和预后进行了大规模的基于人群的描述，魁北克患者和GDV患者之间未发现显著差异。
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