Outcome of patients with intracranial relapse enrolled on national Wilms Tumor Study Group clinical trials

Pediatr Blood Cancer. 2017 Jul;64(7):10.1002/pbc.26406. doi: 10.1002/pbc.26406. Epub 2017 Jan 18.


Background: The occurrence of brain metastases (at diagnosis or at relapse) in patients with Wilms tumor is very rare.

Methods: We retrospectively reviewed the clinical characteristics of patients with Wilms tumor and relapse to the brain enrolled on the National Wilms Tumor Studies (NWTSs) 1-5.

Results: Intracranial relapse was documented in 47 patients (0.5%). Of the 45 patients with adequate data, 26 (58%) patients were male. Thirty-eight (84%) patients had favorable histology Wilms tumor. In 30 patients (67%), the appearance of intracranial disease was preceded by relapse at another site. Ten patients did not have any disease-directed therapy. Surgical resection was attempted in 15 patients; gross total resection was achieved in 11 patients. Twenty-nine patients received brain irradiation; the median dose was 3,000 cGy (range 1,080-4,000 cGy). Twenty-seven patients received chemotherapy. The 5-year overall survival from the time of intracranial relapse was 28.7% (95% confidence interval: 14.4-43.1%). Nine patients (all favorable histology Wilms tumor) were alive with a median follow-up from brain relapse of 140 months (range 35-381 months). All nine survivors received radiation therapy, eight received chemotherapy, and four underwent surgery (two gross total resection, two partial resection). The overall survival after brain metastases of the NWTS-5 patients was significantly higher than the overall survival of the NWTS 1-4 patients (P value = 0.029, log-rank test).

Conclusions: Patients with Wilms tumor recurrence involving the brain may have durable survival, particularly those treated in recent years. Multimodality therapy including radiation and chemotherapy should be considered for these patients.

Keywords: Wilms tumor; central nervous system; chemotherapy; radiation; surgery.

MeSH terms

  • Adolescent
  • Brain Neoplasms / epidemiology*
  • Brain Neoplasms / secondary*
  • Brain Neoplasms / therapy*
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Disease-Free Survival
  • Female
  • Humans
  • Infant
  • Kidney Neoplasms / pathology*
  • Male
  • Retrospective Studies
  • Wilms Tumor / secondary*