间变性组织学肾母细胞瘤的治疗：第五届全国肾母细胞瘤研究结果

Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study.

作者信息

Dome Jeffrey S, Cotton Cecilia A, Perlman Elizabeth J, Breslow Norman E, Kalapurakal John A, Ritchey Michael L, Grundy Paul E, Malogolowkin Marcio, Beckwith J Bruce, Shamberger Robert C, Haase Gerald M, Coppes Max J, Coccia Peter, Kletzel Morris, Weetman Robert M, Donaldson Milton, Macklis Roger M, Green Daniel M

机构信息

Department of Hematology/Oncology, St Jude Children's Research Hospital, Memphis, TN 38105, USA.

出版信息

J Clin Oncol. 2006 May 20;24(15):2352-8. doi: 10.1200/JCO.2005.04.7852.

DOI:10.1200/JCO.2005.04.7852

PMID:16710034

Abstract

PURPOSE

An objective of the fifth National Wilms' Tumor Study (NWTS-5) was to evaluate the efficacy of treatment regimens for anaplastic histology Wilms' tumor (AH).

PATIENTS AND METHODS

Prospective single-arm studies were conducted. Patients with stage I AH were treated with vincristine and dactinomycin for 18 weeks. Patients with stages II to IV diffuse AH were treated with vincristine, doxorubicin, cyclophosphamide, and etoposide for 24 weeks plus flank/abdominal radiation.

RESULTS

A total of 2,596 patients with Wilms' tumor were enrolled onto NWTS-5, of whom 281 (10.8%) had AH. Four-year event-free survival (EFS) and overall survival (OS) estimates for assessable patients with stage I AH (n = 29) were 69.5% (95% CI, 46.9 to 84.0) and 82.6% (95% CI, 63.1 to 92.4). In comparison, 4-year EFS and OS estimates for patients with stage I favorable histology (FH; n = 473) were 92.4% (95% CI, 89.5 to 94.5) and 98.3% (95% CI, 96.4 to 99.2). Four-year EFS estimates for patients who underwent immediate nephrectomy with stages II (n = 23), III (n = 43), and IV (n = 15) diffuse AH were 82.6% (95% CI, 60.1 to 93.1), 64.7% (95% CI, 48.3 to 77.7), and 33.3% (95% CI, 12.2 to 56.4), respectively. OS was similar to EFS for these groups. There were no local recurrences among patients with stage II AH. Four-year EFS and OS estimates for patients with bilateral AH (n = 29) were 43.8% (95% CI, 24.2 to 61.8) and 55.2% (95% CI, 34.8 to 71.7), respectively.

CONCLUSION

The prognosis for patients with stage I AH is worse than that for patients with stage I FH. Novel treatment strategies are needed to improve outcomes for patients with AH, especially those with stage III to V disease.

摘要

目的

第五次全国肾母细胞瘤研究（NWTS - 5）的一个目标是评估间变性组织学肾母细胞瘤（AH）治疗方案的疗效。

患者与方法

进行前瞻性单臂研究。I期AH患者接受长春新碱和放线菌素D治疗18周。II至IV期弥漫性AH患者接受长春新碱、阿霉素、环磷酰胺和依托泊苷治疗24周并加用侧腹/腹部放疗。

结果

共有2596例肾母细胞瘤患者纳入NWTS - 5，其中281例（10.8%）为AH。I期AH可评估患者（n = 29）的4年无事件生存率（EFS）和总生存率（OS）估计分别为69.5%（95%CI，46.9至84.0）和82.6%（95%CI，63.1至92.4）。相比之下，I期预后良好组织学（FH；n = 473）患者的4年EFS和OS估计分别为92.4%（95%CI，89.5至94.5）和98.3%（95%CI，96.4至99.2）。II期（n = 23）、III期（n = 43）和IV期（n = 15）弥漫性AH患者接受即刻肾切除术后的4年EFS估计分别为82.6%（95%CI, 60.1至93.1）、64.7%（95%CI，48.3至77.7）和33.3%（95%CI，12.2至56.4）。这些组的OS与EFS相似。II期AH患者中无局部复发。双侧AH患者（n = 29）的4年EFS和OS估计分别为43.8%（95%CI，24.2至61.8）和and 55.2%（95%CI，34.8至71.7）。