NATIONAL WILMS TUMOR STUDY--5

现代治疗方法极大地提高了存活率 患有肾母细胞瘤的儿童。进行的随机临床试验 国家肾母细胞瘤研究小组证明，90%的儿童 具有I、II期良好组织学特征的Wilms瘤可以治疗 仅用肾切除和联合化疗成功 长春新碱和放线菌素，同时接受肾切除术， 长春新碱的放射治疗和三联化疗， 放线菌素和阿霉素联合应用可获得长期无复发生存 85%的患者为III、IV期，组织学良好的肾母细胞瘤。 儿童间变性肾母细胞瘤和透明细胞瘤的预后 肾肉瘤(CCSK)随着阿霉素的加入而得到改善 (CCSK)与阿霉素、环磷酰胺(间变性)联合治疗 长春新碱和放线菌素。临床和病理特征有 在初次诊断时不允许进行身份识别 在每个阶段内注定会复发的患者。这个身份证明是 有必要允许更积极的治疗方案的管理 一小群注定要复发而不暴露的患者 使用现有治疗方法预后良好的患者 与更激进的化疗相关的额外风险 养生法。在一项初步研究中确定了生物变量 可能与复发风险相关。我们将在一份确认书中确定 研究条件：(1)16q或1p染色体区域杂合性缺失； 和/或(2)用流式细胞仪测定的DNA含量增加 慢性粒细胞白血病儿童两年无复发生存率显著下降 组织学良好的肾母细胞瘤。我们将评估这一计划的有效性 化疗方案包括更大剂量强度的 环磷酰胺加用依托泊苷治疗卵巢癌 儿童肾间变性肾母细胞瘤和透明细胞肉瘤。 我们将评估依托泊苷和卡铂的联合作用。 和环磷酰胺治疗儿童肾脏横纹肌样瘤。我们 将采用统一的策略来治疗复发的儿童 以确定这些生物因素是否与糖尿病的风险有关 复发也预示着对恢复治疗的反应。大多数人的生存 治疗肾母细胞瘤的儿童允许研究遗传和治疗 与疾病的病因和/或发生有关的因素 治疗的并发症。我们将与所有患者保持联系，以 允许研究肾母细胞瘤的遗传模式， 治疗对生育力和妊娠结局的影响 充血性心力衰竭与二次恶性肿瘤的发生 肿瘤。

Modern treatment methods have led to major improvements in survival rates of children with Wilms tumor. Randomized clinical trials conducted by the National Wilms Tumor Study Group have demonstrated that 90% of children with stage I and II favorable histology Wilms tumor can be treated successfully with only nephrectomy and combination chemotherapy consisting of vincristine and dactinomycin, while treatment with nephrectomy, radiation therapy and three-drug chemotherapy with vincristine, dactinomycin and doxorubicin results in long-term relapse-free survival of 85% of patients with stage III and IV, favorable histology Wilms tumor. The prognosis for children with anaplastic Wilms tumor and clear cell sarcoma of the kidney (CCSK) has improved with the addition of doxorubicin (CCSK) and doxorubicin and cyclophosphamide (anaplasia) to the combination of vincristine and dactinomycin. Clinical and pathological features have not allowed identification at the time of initial diagnosis of those patients destined to relapse within each stage. This identification is necessary to allow administration of more aggressive treatment regimens to the small group of patients who are destined to relapse without exposing those patients with an excellent prognosis using current treatment methods to the additional risk associated with more aggressive chemotherapeutic regimens. Biological variables have been identified in a pilot study which may correlate with risk of relapse. We will determine in a confirmatory study if: (1) loss of heterozygosity for chromosomal regions 16q or 1p; and/or (2) increased DNA content determined using flow cytometry predicts significantly worse two-year relapse-free survival in children with favorable histology Wilms tumor. We will evaluate the effectiveness of chemotherapy regimens which include greater dose intensity of cyclophosphamide and the addition of etoposide for the treatment of children with anaplastic Wilms tumor and clear cell sarcoma of the kidney. We will evaluate the activity of the combination of etoposide, carboplatin and cyclophosphamide in children with rhabdoid tumor of the kidney. We will employ a uniform strategy for the treatment of children who relapse to determine if those biological factors associated with the risk of relapse also predict response to retrieval therapy. The survival of most children treated for Wilms tumor allows the study of genetic and treatment factors associated with the etiology of the disease and/or the occurrence of complications of therapy. We will maintain contact with all patients to allow study of the pattern of inheritance of Wilms tumor, the effect of therapy on fertility and pregnancy outcome, the risk factors for congestive heart failure and for the occurrence of second malignant tumors.