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A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): A Children's Oncology Group prospective study

The Lancet Oncology Dec 11, 2019

Spunt SL, Million L, Chi YY, et al. - Researchers performed this prospective analysis to construct a risk stratification system for paediatric non-rhabdomyosarcoma soft-tissue sarcoma (NRSTS), based on known prognostic factors. They also evaluated it in the context of risk-adapted treatment for young patients with NRSTS. The participants were enrolled in 159 hospitals in three countries and were younger than 30 years. Allocation of each patient to one of three risk groups and one of four treatment groups was done. The risk groups were defined as: low (non-metastatic R0 or R1 low-grade, or ≤ 5 cm R1 high-grade tumour); intermediate (non-metastatic R0 or R1 > 5 cm high-grade, or unresected tumour of any size or grade); or high (metastatic tumour). The treatment groups included surgery alone, radiotherapy, chemoradiotherapy, and neoadjuvant chemoradiotherapy. There were 529 evaluable patients. In the low-risk group, the 5-year event-free survival and overall survival were estimated to be 88·9% and 96·2% at a median follow-up of 6·5 years, these were 65·0% and 79·2% in the intermediate-risk group and 21·2% and 35·5% in the high-risk group. Findings revealed the utility of pretreatment clinical characteristics for effective determination of treatment failure risk and stratification of young patients with NRSTS for risk-adapted treatment. Cure can be achieved in most low-risk cases without adjuvant therapy, thereby avoiding known long-term treatment complications. For intermediate-risk and high-risk patients, survival continued to be suboptimal and there is a need for new therapies.
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