Successful treatment of infants with localized neuroblastoma based on their MYCN status

Successful treatment of infants with localized neuroblastoma based on their MYCN status
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DOI:
10.1007/s10147-012-0391-y
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发表时间:
2013-06-01
影响因子:
3.3
通讯作者:
Sawada, Tadashi
Sawada, Tadashi
中科院分区:
医学3区
文献类型:
--
作者:
Iehara, Tomoko;Hamazaki, Minoru;Sawada, Tadashi

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本研究的目的是评估手术后化疗的有效性与局限性神经母细胞瘤无MYCN扩增(MNA)的婴儿,并确定是否使用MNA的风险分类是合理的。1期和2A/2B期可切除的患者仅接受手术切除治疗。无MNA的3期不可切除患者在手术切除前接受6个周期的方案A或3个周期的方案A加3个周期的方案C2;方案A由低剂量环磷酰胺和长春新碱组成,方案C由环磷酰胺、长春新碱和吡拉托溴铵组成。随机选择可切除和不可切除的患者接受术后化疗。MNA患者接受D2方案强化化疗,部分患者接受大剂量化疗联合干细胞移植,1期和2A/2B期无MNA患者的5年无事件生存率(5-EFS)分别为97.2%和89.0%(p = 0.02)。共有31名没有MNA的3期患者接受了术后化疗,30名患者没有接受化疗。这两组的5-EFS率(分别为96.0%和96.2%)无显著差异(p = 0.869)。局限性MNA患者5-EFS率为50.0%,而非MNA患者5-EFS率为95.0%(P < 0.001),因此,局限性MNA患者术后无需化疗。使用MNA的这种治疗策略被认为适用于婴儿。
The aim of this study was to evaluate the effectiveness of post-surgical chemotherapy for infants with localized neuroblastoma without MYCN amplification (MNA), and determine whether risk classification using MNA is reasonable.Four hundred and fourteen eligible patients were registered between 1998 and 2004. Resectable patients in stage 1 and 2A/2B were treated by surgical resection only. Unresectable patients in stage 3 without MNA received either 6 cycles of regimen A or 3 cycles of regimen A plus 3 cycles of regimen C2; regimen A consisted of low doses of cyclophosphamide and vincristine and regimen C consisted of cyclophosphamide, vincristine and pirarubicin before surgical resection. The resectable and unresectable patients were randomly selected to receive post-surgical chemotherapy. The patients with MNA received intensive chemotherapy regimen D2, consisting of cyclophosphamide, vincristine, pirarubicin and cisplatin, and some of them received high-dose chemotherapy with stem cell transplantation.The 5-year event-free survival (5-EFS) rates of stage 1 and 2A/2B patients without MNA were 97.2 and 89.0% respectively (p = 0.02). A total of 31 patients in stage 3 without MNA received post-surgical chemotherapy, and 30 patients did not. The 5-EFS rates of these two groups (96.0 and 96.2%, respectively) were not significantly different (p = 0.869). The 5-EFS rate for localized patients with MNA (n = 6) was 50.0%, and that of patients without MNA was 95.0% (p < 0.001).Post-surgical chemotherapy was therefore unnecessary for localized patients without MNA. This treatment strategy using MNA is considered to be appropriate in infants.