Impact of Radiation Therapy on Malignant Rhabdoid Tumor of the Kidney: A National Cancer Database Analysis.
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BACKGROUND: Malignant rhabdoid tumor of the kidney (MRTK) is a rare and aggressive tumor, primarily affecting infants and young children. Intensive multimodal treatment is standard, entailing chemotherapy, surgery, and radiation therapy (RT). Although previous studies have demonstrated the importance of chemotherapy and surgery, the impact of RT on patient outcomes is unclear. METHODS: Patients diagnosed with MRTK from 2004 to 2019 were analyzed from the National Cancer Database. Patients who did not receive surgery or chemotherapy or died within 30 days after surgery were excluded. Kaplan-Meier survival analyses were conducted to evaluate overall survival (OS) of the entire cohort, stratified by age, metastatic disease, and RT utilization. Log-rank tests were used to compare survival distributions. RESULTS: The cohort included 103 patients. Median age was <1 year (range <1-15, interquartile range: 0-1 year). Forty-eight (47%) patients presented with metastatic disease at diagnosis. Sixty-six (64%) patients received RT. One- and three-year OS were 55% and 45%, respectively. Median OS for the entire cohort was 12.2 months. Patients aged <1 year exhibited significantly worse 3-year OS compared to those aged ≥1 year (28% vs. 64%, p < 0.001). Metastatic disease was associated with worse 3-year OS compared to localized disease (25% vs. 62%, p < 0.001). Patients who received RT demonstrated significantly better 3-year OS than those who did not receive RT (58% vs. 19%, p < 0.001). In patients with localized disease, RT to the primary tumor bed was associated with improved 3-year OS compared to no RT (74% vs. 33%, p = 0.008). Among patients with metastatic disease, treatment with RT to either primary and/or metastatic site was also associated with a 3-year OS benefit compared to no RT (38% vs. 10%, p < 0.001). CONCLUSION: Although MRTK remains highly lethal in infants and particularly those with metastatic disease, RT was independently associated with improved survival in patients with and without metastatic disease.