Incidence, Survival, and Treatment of Localized and Metastatic Neuroblastoma in Germany 1979-2015.

Incidence, Survival, and Treatment of Localized and Metastatic Neuroblastoma in Germany 1979-2015.
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DOI:
10.1007/s40272-017-0251-3
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发表时间:
2017-12
期刊:
Paediatric drugs
影响因子:
--
通讯作者:
Lampert F
Lampert F
中科院分区:
其他
文献类型:
--
作者:
Berthold F;Spix C;Kaatsch P;Lampert F

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文献中缺乏对神经细胞瘤疾病全谱的全面临床长期调查。我们的目的是描述德国神经母细胞瘤患者的发病率、风险概况、治疗方法和结局,包括所有临床阶段和风险组。回顾性分析1979年至2015年间参加6个连续国家试验之一的神经母细胞瘤患者的流行病学、临床和结局资料。在国家儿童癌症登记处已知的所有德国神经母细胞瘤患者中,97%的人参加了其中一项试验。神经母细胞瘤的绝对发病率略有上升,而诊断时的中位年龄有所下降。除了筛查期(1995-2000)外,乳酸脱氢酶(LDH)、铁蛋白、1p染色体和MYCN癌基因的危险因素基本保持不变,除了试验NB97(27%)和NB2004(35%)之间年龄≥18个月的患者在4期MYCN扩增增加。1-3期神经母细胞瘤患者的10年总生存率从83%增加到91%,4S期从80%增加到85%,年龄≥18个月的4期从2%增加到38%。1-3期患者从未接受化疗的比例(无论是一线患者还是复发患者)从35%增加到60%。总体人群以及年龄≥18个月的4期患者的原发肿瘤宏观完全手术切除比例下降。化疗反应对结果的影响与试验有关。在方案治疗期间,年龄≥18个月的4期患者中毒性死亡的总比例为6%,低/中危患者为2%。在年龄≥18个月的4期患者中,最常见的晚期后遗症是肾功能障碍、甲状腺功能减退、严重听力障碍和二次恶性肿瘤。从这项超过37年的调查中获得的发病率、风险概况和生存率的数据为未来神经母细胞瘤亚群的研究提供了有用的视角。本文的在线版本(doi:10.1007/s40272-017-0251-3)包含补充材料,可供授权用户使用。
A comprehensive clinical long-term survey over the complete spectrum of neuroblatoma disease is lacking in the literature. Our objective was to describe the incidence, risk profiles, therapies, and outcomes for the total cohort of German patients with neuroblastoma including all clinical stages and risk groups. Epidemiological, clinical, and outcome data of neuroblastoma patients who participated in one of the six consecutive national trials between 1979 and 2015 were analyzed retrospectively. Of all German neuroblastoma patients known to the national childhood cancer registry, ninety seven percent enrolled in one of the trials. The absolute neuroblastoma rate has increased slightly, whereas the median age at diagnosis has decreased. Except for the screening period (1995–2000), the risk factors lactate dehydrogenase (LDH), ferritin, chromosome 1p, and the MYCN oncogene have remained largely constant, with the exception of an increase in MYCN amplification at stage 4 for those aged ≥18 months between trials NB97 (27%) and NB2004 (35%). The 10-year overall survival increased in patients with stage 1–3 neuroblastoma from 83 to 91%, for stage 4S from 80 to 85%, and for stage 4 aged ≥18 months from 2 to 38%. The fraction of patients in stages 1–3 who never received chemotherapy (neither for frontline nor at recurrence) increased from 35 to 60%. The proportion of macroscopically complete surgical resections of the primary tumor decreased for the total population as well as for patients with stage 4 aged ≥18 months. The impact of chemotherapy response on the outcome was trial dependent. The overall proportion of toxic death during the time of the protocol therapy was 6% for stage 4 patients aged ≥18 months and 2% for low-/intermediate-risk patients. The most frequently reported late sequelae in stage 4 patients aged ≥18 months were renal dysfunctions, hypothyroidism, major hearing impairment, and second malignancies. The body of data for incidences, risk profiles, and survival rates from this survey of more than 37 years provides a useful perspective for future studies on neuroblastoma sub-cohorts. The online version of this article (doi:10.1007/s40272-017-0251-3) contains supplementary material, which is available to authorized users.
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发表时间: 1993-08-01
影响因子: 45.3
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