Improved Survival of Children With Neuroblastoma Between 1979 and 2005: A Report of the Italian Neuroblastoma Registry
Improved Survival of Children With Neuroblastoma Between 1979 and 2005: A Report of the Italian Neuroblastoma Registry
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DOI：
10.1200/jco.2009.24.8351
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发表时间：
2010-05-10
影响因子：
45.3
通讯作者：
De Bernardi, Bruno
中科院分区：
文献类型：
--
作者：
Haupt, Riccardo;Garaventa, Alberto;De Bernardi, Bruno
PurposeTo描述治疗，临床过程，和一个队列的意大利患者与neuroblastoma.Patients和MethodsThe研究包括从1979年至2005年诊断的2,216名儿童（年龄0至14岁）的数据生存。总生存期（OS）分析了临床和生物学特征的表现和诊断时期：1979年至1984年，1985年至1991年，1992年至1998年，1999年至2005年。第二个恶性肿瘤（SMN）的相对风险进行了评估的标准化发病率比（SIR），与意大利人口选定为referent.ResultsYearly患者增加随着时间的推移，从58至102。年龄为0至17个月的患者占总人群的45.6%，其发生率随时间从36.5%增加至48.5%。1期患者的发生率随时间从5.8%增加至23.2%。共有898例患者（40.5%）发生疾病进展或复发，19例患者发生SMN，2例患者发生骨髓增生异常。20年时SMN的累积风险为7.1%，SIR为8.4（95% CI，5.1 - 13.2）。共有858例患者（39%）死亡（779例疾病，71例毒性，6例SMN，2例肿瘤无关手术并发症）。10年OS为55.3%（95% CI，53.0%至57.6%），随时间从34.9%增加至65.0%;女性和诊断时年龄为0至17个月、原发于腹外、1期和2期疾病的患者的OS显著更好。1期和3期患者的OS随时间显著改善。在第4阶段疾病的患者，改善之间发生的第一和第二次队列（6.7%v 23.5%），但不是after.ConclusionThe儿童神经母细胞瘤的结果逐步改善。长期存活者有很大的SMN风险。
PurposeTo describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma.Patients and MethodsThe study includes data from 2,216 children (age 0 to 14 years) diagnosed between 1979 and 2005. Overall survival (OS) was analyzed by clinical and biologic features at presentation and periods of diagnosis: 1979 to 1984, 1985 to 1991, 1992 to 1998, and 1999 to 2005. The relative risk of second malignant neoplasm (SMN) was assessed by the standardized incidence ratio (SIR), with the Italian population selected as referent.ResultsYearly patient accrual increased over time from 58 to 102. Patients age 0 to 17 months represented 45.6% of the total population, and their incidence increased over time from 36.5% to 48.5%. The incidence of stage 1 patients increased over time from 5.8% to 23.2%. A total of 898 patients (40.5%) developed disease progression or relapse, 19 patients developed SMN, and two patients developed myelodysplasia. The cumulative risk of SMN at 20 years was 7.1%, for an SIR of 8.4 (95% CI, 5.1 to 13.2). A total of 858 patients (39%) died (779 of disease, 71 of toxicity, six of SMN, and two of tumor-unrelated surgical complications). Ten-year OS was 55.3% (95% CI, 53.0% to 57.6%) and increased over time from 34.9% to 65.0%; it was significantly better for females and patients age 0 to 17 months at diagnosis, with extra-abdominal primary, and stage 1 and 2 disease. OS improved significantly over time in stage 1 and 3 patients. In patients with stage 4 disease, the improvement occurred between the first and second time cohorts (6.7% v 23.5%), but not afterward.ConclusionThe outcome of children with neuroblastoma has progressively improved. Long-term survivors bear a significant risk of SMN.