Rhabdoid tumour associated with poor outcomes among youths with high-risk renal tumours
In young people with high-risk renal tumours (HRRT), survival is poor in the presence of rhabdoid tumour (RTK) and favourable in the presence of clear cell sarcoma (CCSK), according to a study.
The study included 349 patients aged ≤30 years who were diagnosed with anaplastic Wilms tumour (AWT; 38.1 percent), CCSK (34.4 percent) or RTK (27.5 percent). Compared with those in the AWT or CCSK group, patients in the RTK group were less likely to undergo surgery (94 percent vs 99 percent vs 77.1 percent, respectively; p<0.001) and to receive chemotherapy (96.2 percent vs 95 percent vs 84.4 percent, respectively; p=0.013) or radiation therapy (81.2 percent vs 86.7 percent vs 52.1 percent, respectively; p<0.001).
Estimated 5-year overall survival (OS) was 76.1 percent (67.9–84.4) in the AWT group, 92.7 percent (87.4–97.9) in the CCSK group and 33.5 percent (23.1–43.9) in the RTK group (p<0.001).
Multivariate Cox regression analysis showed that worse OS was significantly associated with AWT (hazard ratio [HR], 3.372; p=0.032) and RTK histology (HR, 12.595; p<0.001), whereas improved OS was associated with receipt of radiation therapy (HR, 0.43; p=0.006). No such associations were observed for LN positivity, margin status, and undergoing surgery.
When HRRTs were analysed individually, factors associated with OS included undergoing surgery for AWT (HR, 0.308; p=0.031), and both undergoing surgery (HR 0.209, p=0.007) and receipt of radiation therapy for RTK (HR, 0.411; p=0.008).
According to researchers, adjuvant radiation, while significantly associated with improved outcomes for all HRRTs, may be underutilized in patients at the highest risk, namely those with RTK. This indicates a potential for future improvement in the care of this population.
Additional investigation into the specific biology of RTKs is needed to allow for more precision and targeted therapies, which should translate to gains in the prognosis of such tumours, researchers added.