Corey S J, Andersen J W, Vawter G F, Lack E E, Sallan S E
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA 02115.
Cancer. 1991 Sep 1;68(5):970-4. doi: 10.1002/1097-0142(19910901)68:5<970::aid-cncr2820680510>3.0.co;2-5.
Combined modality treatment has resulted in cure rates of approximately 80% for children with Wilms' tumor. According to the National Wilms' Tumor Studies (NWTS), a group of patients with histologic features of anaplasia or sarcomatous Wilms' tumors (malignant rhabdoid tumors and clear cell sarcomas) were less responsive to vincristine and actinomycin. The survival rate of patients in this group with unfavorable histologic conditions was 54% compared with 90% for those with favorable histologic conditions. We have reviewed 80 consecutive cases of Wilms' tumor treated with a minimum follow-up interval of 5 years. Two pathologists independently reviewed all histologic specimens that were initially classified as having unfavorable histologic conditions and specimens from children with favorable histologic conditions who subsequently relapsed. One of 13 children with favorable histologic conditions had recurrent disease that was found to have unfavorable histologic conditions on rereview. All five patients with sarcomatous Wilms' tumor had a rapidly progressive course. Treatment of eight children with anaplastic Wilms' tumor with vincristine, actinomycin, cyclophosphamide, and abdominal radiation resulted in good disease-free and overall survival rates (5-year survival rate, 87.5%) that were not significantly different from children with tumors having favorable histologic conditions (5-year survival rate, 94%). All children with sarcomatous histologic conditions, however, did not to respond.
综合治疗使肾母细胞瘤患儿的治愈率达到了约80%。根据国家肾母细胞瘤研究（NWTS），一组具有间变或肉瘤样肾母细胞瘤组织学特征（恶性横纹肌样瘤和透明细胞肉瘤）的患者对长春新碱和放线菌素的反应较差。组织学条件不利的该组患者的生存率为54%，而组织学条件良好的患者为90%。我们回顾了80例连续接受治疗且最短随访间隔为5年的肾母细胞瘤病例。两名病理学家独立复查了所有最初被归类为组织学条件不利的组织标本以及组织学条件良好但随后复发的儿童的标本。13名组织学条件良好的儿童中有1名复发疾病，复查时发现其组织学条件不利。所有5例肉瘤样肾母细胞瘤患者病程进展迅速。用长春新碱、放线菌素、环磷酰胺和腹部放疗治疗8例间变性肾母细胞瘤患儿，其无病生存率和总生存率良好（5年生存率为87.5%），与组织学条件良好的肿瘤患儿（5年生存率为94%）无显著差异。然而，所有具有肉瘤样组织学条件的儿童均无反应。
