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

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

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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Anaplasia
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Child
  • Child, Preschool
  • Cyclophosphamide
  • Dactinomycin / administration & dosage
  • Doxorubicin / administration & dosage
  • Etoposide / administration & dosage
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Kidney / pathology*
  • Kidney Neoplasms / pathology
  • Kidney Neoplasms / therapy*
  • Male
  • Neoplasm Staging
  • Nephrectomy
  • Prognosis
  • Prospective Studies
  • Radiotherapy
  • Rhabdoid Tumor / pathology
  • Rhabdoid Tumor / therapy
  • Sarcoma, Clear Cell / pathology
  • Sarcoma, Clear Cell / therapy
  • Survival Analysis
  • Vincristine / administration & dosage
  • Wilms Tumor / pathology
  • Wilms Tumor / therapy*

Substances

  • Dactinomycin
  • Vincristine
  • Etoposide
  • Doxorubicin
  • Cyclophosphamide