Long-Term Outcomes Among Survivors of Wilms Tumor

Get Permission

In an analysis from the Childhood Cancer Survivor Study (CCSS) reported in the Journal of Clinical Oncology, Weil et al found that survivors of unilateral Wilms tumor were at an increased risk for severe chronic health conditions.

Study Details

The study involved 2,008 5-year survivors diagnosed between 1970 and 1999, with a median age at diagnosis of 3.2 years and median age at follow-up of 27.8 years. All survivors underwent unilateral nephrectomy, and 98% received chemotherapy. The cumulative incidence of late mortality (> 5 years from diagnosis) and grade 3 to 5 chronic health conditions were assessed and evaluated by treatment, including nephrectomy with vincristine and dactinomycin, vincristine/dactinomycin plus doxorubicin and abdominal radiotherapy with or without whole-lung radiotherapy, and receipt of four or more chemotherapy agents. Follow-up for mortality was through December 2017. Comparisons were made with 5,006 siblings of CCSS survivors (not limited to Wilms tumor survivors) and with the general U.S. population matched for age, sex, and calendar year.

Key Findings

Among the 2,008 survivors, death occurred in 142 (standardized mortality ratio [SMR] for all-cause mortality vs general population = 2.9, 95% confidence interval [CI] = 2.5–3.5). The 35-year cumulative incidence of death was 7.8% (95% CI = 6.3%–9.2%). The 35-year cumulative incidence of any grade 3 to 5 chronic health condition was 34.1% (95% CI = 30.7%­–37.5%) among survivors vs 14.8% (95% CI = 13.4%–16.2%) among siblings (rate ratio [RR] = 3.0, 95% CI = 2.6–3.5).

Survivors treated with vincristine/dactinomycin alone in addition to nephrectomy did not have a significantly elevated risk for all-cause late mortality vs the general population (SMR = 1.0, 95% CI = 0.5–1.7). Compared with siblings, these survivors were at a somewhat increased risk of grade 3 to 5 chronic health conditions (RR = 1.5, 95% CI = 1.1–2.0), but had a markedly increased risk for intestinal obstruction (RR = 9.4, 95% CI = 3.9–22.2) and kidney failure (RR = 11.9, 95% CI = 4.2–33.6). No increased risks of heart failure or premature ovarian insufficiency were observed. Compared with risk among survivors who received only vincristine/dactinomycin in addition to nephrectomy, magnitudes of risk for grade 3 to 5 chronic health conditions—including intestinal obstruction, kidney failure, premature ovarian insufficiency, and heart failure—increased by treatment group intensity.

The investigators concluded, “With approximately 40% of patients with newly diagnosed Wilms tumor currently treated with [vincristine/dactinomycin] alone, the burden of late mortality/morbidity in future decades is projected to be lower than that for survivors from earlier eras. Nevertheless, the risk of late effects such as intestinal obstruction and kidney failure was elevated across all treatment groups, and there was a dose-dependent increase in risk for all grade 3 to 5 chronic health conditions by treatment group intensity.”

Brent R. Weil, MD, MPH, of the Department of Surgery, Boston Children’s Hospital and Harvard Medical School, is the corresponding author for the Journal of Clinical Oncology article.

Disclosure: The study was supported by grants from the National Cancer Institute and American Lebanese Syrian Associated Charities. For full disclosures of the study authors, visit

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.