Biologic factors determine prognosis in infants with stage IV neuroblastoma: A prospective Children's Cancer Group study
Biologic factors determine prognosis in infants with stage IV neuroblastoma: A prospective Children's Cancer Group study
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DOI：
10.1200/jco.2000.18.6.1260
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发表时间：
2000-03-01
影响因子：
45.3
通讯作者：
Matthay, KK
中科院分区：
文献类型：
--
作者：
Schmidt, ML;Lukens, JN;Matthay, KK
目的：一项儿童癌症组前瞻性研究CCG-3881已经完成，以确定通过生物学特征更准确地预测预后是否可以识别需要不同治疗强度的IV期神经母细胞瘤婴儿亚型。患者和方法：1989年6月至1995年8月登记的婴儿134例，中位随访时间为47.1个月(范围0~88个月)。检测的生物学因素包括肿瘤MYCN拷贝数、岛田组织病理学分类、血清铁蛋白和骨髓免疫细胞学(敏感性，每10(5)个骨髓细胞中有一个肿瘤细胞)。接受CCG-3881化疗的患者(n=116)接受四种药物(顺铂、环磷酰胺、阿霉素和依托泊苷)化疗9个月，同时手术和局部放射治疗残留病变。结果：134例患儿的3年无事件生存率(EFS)和总生存率(Mean+/-SD)分别为63%+/-5%和71%+/-5%。无MYCN扩增的患者3年EFS为93%+/-4%，而MYCN扩增的患者3年EFS为10%+/-7%(P<；.0001)。其他生物学特征在单因素分析中均有预后意义，但按MYCN拷贝数分层后不具有预测意义。结论：小于1岁的诊断为IV期NBL的婴儿与大于或等于1岁的儿童相比，预后明显改善。未扩增的MYCN肿瘤识别具有93%+/-4%EFS的ct组婴儿，而扩增的MYCN拷贝数清楚地识别尽管进行密集化疗但不太可能存活的患者，(C)美国临床肿瘤学会2000年。
Purpose: A prospective Children's Cancer Group study, CCG-3881, has been completed to determine if a more accurate prediction of prognosis by biologic features can identify subgroups of infants with stage IV neuroblastoma (NBL) who require differing intensities of treatment.Patients and Methods: One hundred thirty-four infants were registered from June 1989 to August 1995, with a median follow-up of 47.1 months (range, 0 to 88 months). The biologic factors examined were tumor MYCN copy number, Shimada histopathologic classification, serum ferritin, and bone marrow immunocytology (sensitivity, one tumor cell per 10(5) bone marrow cells). patients treated on CCG-3881 (n = 116) received four-drug chemotherapy for 9 months (cisplatin, cyclophosphamide, doxorubicin, and etoposide), with surgery and local radiation to residual disease. After January 1991, all subsequent infants with tumor MYCN amplification (9 = 18) were transferred after one cycle of therapy to the high-risk CCG-3891 protocol (open January 1991 to April 1996) for more intensive treatment,Results: The 3-year event-free survival (EFS) and overall survival (mean +/- SD) for the 134 infants were 63% +/- 5% and 71% +/- 5%, respectively. patients whose tumors were without MYCN amplification had a 93% +/- 4% 3-year EFS, whereas those with amplified MYCN had a 10% +/- 7% 3-year EFS (P < .0001). Each of the other biologic features studied had prognostic significance in univariate analysis but not after stratifying by MYCN copy number.Conclusion: infants less: than 1 year of age at diagnosis with stage IV NBL have a much improved outcome compared with children greater than or equal to 1 year of age. Nonamplified MYCN tumors identify ct group of infants with a 93% +/- 4% EFS, whereas amplified MYCN copy number clearly identifies patients who are unlikely to survive despite intensive chemotherapy, (C) 2000 by American Society of Clinical Oncology.