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成人神经母细胞瘤:基于监测、流行病学与最终结果(SEER)数据的发病率及生存分析

Neuroblastoma in adults: Incidence and survival analysis based on SEER data.

作者信息

Esiashvili Natia, Goodman Michael, Ward Kevin, Marcus Robert B, Johnstone Peter A S

机构信息

Department of Radiation Oncology, Emory University School of Medicine, Atlanta, Georgia 30322, USA.

出版信息

Pediatr Blood Cancer. 2007 Jul;49(1):41-6. doi: 10.1002/pbc.20859.

DOI:10.1002/pbc.20859
PMID:16628557
Abstract

PURPOSE

Clinical data on survival outcomes of adult patients (defined as 20 years of age and older) with neuroblastoma are scarce due to the rarity of the disease. Small single institution reports have described worse outcomes for adults than pediatric patients.

MATERIALS/METHODS: Data from the public access Surveillance Epidemiology and End Results database were reviewed for a 30-year period between 1973 and 2002 for the diagnosis of neuroblastoma. Analyses compared frequency and survival rates of neuroblastoma among children and adults with particular focus on the incidence, case characteristics, and long-term survival of the adult patients.

RESULTS

The observed 3- and 5-year survival rates were lowest among adult patients (45.9% and 36.3%, respectively), whereas infants fared best with 86.0% 3-year and 84.6% 5-year overall survival. There was a steady decline in the incidence of adult neuroblastoma from 0.47 cases per million per year in 1973-1977 to 0.12 cases per million per year in 1998-2002. When the long-term adult neuroblastoma survival was examined in detail for 1-, 3-, 5-, 7-, and 10-year follow-up periods, there was continuous decrease in survival during the first 7 years after the diagnosis with subsequent stabilization for both observed (OS) and relative survival (RS) rates. There was a notable trend towards improved outcome over the past three decades.

CONCLUSIONS

Adults with neuroblastoma have significantly worse outcome than children. This may be due to tumor biology, more virulent clinical course, or possibly due to the fact that adults are less sensitive or have poor tolerance to pediatric chemotherapy regimens.

摘要

目的

由于神经母细胞瘤疾病罕见,关于成年患者(定义为20岁及以上)神经母细胞瘤生存结局的临床数据稀缺。小型单机构报告描述了成年患者的结局比儿科患者更差。

材料/方法:回顾了公开可用的监测、流行病学和最终结果数据库在1973年至2002年30年间诊断为神经母细胞瘤的数据。分析比较了儿童和成人群体中神经母细胞瘤的发病率和生存率,特别关注成年患者的发病率、病例特征和长期生存情况。

结果

成年患者的3年和5年生存率最低(分别为45.9%和36.3%),而婴儿的情况最佳,3年总生存率为86.0%,5年总生存率为84.6%。成年神经母细胞瘤的发病率从1973 - 1977年的每年每百万0.47例稳步下降至1998 - 2002年的每年每百万0.12例。在对成年神经母细胞瘤进行1年、3年、5年、7年和10年随访期的详细长期生存情况检查时,诊断后的前7年生存率持续下降,随后观察生存率(OS)和相对生存率(RS)均趋于稳定。在过去三十年中存在结局改善的显著趋势。

结论

患有神经母细胞瘤的成年人结局明显比儿童差。这可能是由于肿瘤生物学特性、更具侵袭性的临床病程,或者可能是因为成年人对儿科化疗方案不太敏感或耐受性较差。

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