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Objective:To summarize the clinical features of clear cell sarcoma of the kidney (CCSK) in children and to evaluate the effica-cy and safety of the WT-2009 chemotherapy protocol. Methods:Clinical data of children with CCSK recorded between January 2009 and December 2014 were retrospectively analyzed. Follow-ups were conducted to monitor the postoperative conditions of the pa-tients. Results:Nine cases of CCSK were recruited, and another three cases were excluded for the analysis because of incomplete clini-cal data. All patients achieved complete remission when the treatment was finished. The estimated 3-year overall survival rate was 100％, and the estimated 3-year event-free survival rate was 83.3％. No report is available on the significant side effects associated with this treatment. Conclusion:CCSK is rare in children and is easily misdiagnosed. The chemotherapy based on the WT-2009 proto-col can produce a favorable prognosis and a high tolerance for patients with CCSK. However, the treatment for high-risk patient needs to be further explored, and follow-ups must be intensified.
RESUMEN
Objective To evaluate the clinical outcome of NB09 (China Pediatric Neuroblastoma cooperative group 09) protocol on children with high-risk and ultra-high risk neuroblastoma. Methods The clinical and follow-up data of pa?tients who suffered from high-risk (n=7) and ultra-high risk (n=31) neuroblastomas and admitted in Tumor hospital of Tian?jin Medical University between January 2009 to January 2013 were retrospectively reviewed (27 boys and 11 girls). The age at diagnosis was 19-160 months (median age was 36.5 months). In the high risk group, patients were evaluated and operated after 4 to 6 circles of neoadjuvant chemotherapy. In ultra-high risk group, patient received chemotherapy before and after op?eration, then autologous stem cell transplantation and tumor bed radiotherapy. After chemotherapy, retinoic acid treatment was given to patients in ultra high risk group as in high risk group. Results At the end of treatment, 25 patients achieved complete remission; 5 patients achieved partial remission; 3 patients were in stable disease;5 patients were deteriorating in their conditions which lead to 2 deaths. In total, the response rate reaches upto 86.8%. By the end of follow up, 15 patients had a disease-free-survival, 9 patients survived with tumor, 7 died from recurrence and 7 died from deteriorating conditions. Survival time ranged from 6 to 52 months (median survival 25.5 months). The 1-, 2- and 3-year overall survival were 91.7%, 64.5%and 57.3%respectively. Kaplan-Meier curve and Log-rank test showed no statistical significance between high risk and ultra-high risk neuroblastomas. Conclusion The outcome of NB09 protocol for high risk and ultra-high risk neuroblastoma was preliminary affirmed. It is worthy of further clinical verification.