Clinical analysis of 12 children with clear cell sarcoma of kidney
唐京京 1徐学聚 1白松婷 1王璐 1刘玉峰 1单卫华
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作者信息
1. 郑州大学第一附属医院儿童医院血液肿瘤科，郑州 450052
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摘要
目的 分析儿童肾透明细胞肉瘤(CCSK)的临床特征、治疗及预后。 方法 回顾性病例分析。研究对象为2014年1月至2021年12月郑州大学第一附属医院儿童医院血液肿瘤科收治的12例CCSK患儿，总结其临床特征、诊治过程及随访结果，采用Kaplan-Meier生存分析法计算生存率。 结果 12例患儿中男10例，女2例，发病年龄2.8(1.0～5.7)岁，6例起源于左肾，6例起源于右肾。患儿均先手术完整切除原发瘤灶，Ⅰ期2例，Ⅱ期4例，Ⅲ期3例，Ⅳ期3例；12例患儿均接受相应的化疗方案，6例接受放疗。随访至2023年12月31日，4例死于疾病复发，8例无病生存，随访时间56(19～94)个月，5年无事件生存率为(62±11)%，5年总生存率为(72±9)%。 结论 儿童CCSK好发于男性幼儿，采用根治性手术及术后放化疗的多学科联合治疗，早期分期预后良好，而复发及远处转移者死亡率高。 Objective To analyze the clinical features, treatment and prognosis of clear cell sarcoma of kidney (CCSK) in children. Methods A retrospective case series study was conducted on 12 children with CCSK who were admitted to the Department of Hematology and Oncology, Children′s Hospital, the First Affiliated Hospital of Zhengzhou University from January 2014 to December 2021.The clinical characteristics, diagnosis and treatment process, and follow-up results were summarized, and the survival rate was calculated using the Kaplan-Meier method. Results Among the 12 patients, 10 were males, and 2 were females, with the age of onset of 2.8 (1.0-5.7) years.CCSK originated from the left kidney in 6 patients and from the right kidney in 6 patients.All the patients underwent complete resection of the tumor, including 2 patients in stage Ⅰ, 4 patients in stage Ⅱ, 3 patients in stage Ⅲ, and 3 patients in stage Ⅳ and 12 children received the corresponding chemotherapy regimen, 6 cases received radiotherapy.During the follow-up of 56 (19-94) months to December 31, 2023, 4 cases died of disease recurrence, and 8 cases survived without disease.The 5-year event-free survival rate was (62±11)%, and the 5-year overall survival rate was (72±9)%. Conclusions Pediatric CCSK is more common in male children.The combination of radical surgery and postoperative chemoradiotherapy offers a good prognosis in the early stage, while the mortality of patients due to recurrence and distant metastasis is high.
Abstract
Objective To analyze the clinical features, treatment and prognosis of clear cell sarcoma of kidney (CCSK) in children. Methods A retrospective case series study was conducted on 12 children with CCSK who were admitted to the Department of Hematology and Oncology, Children′s Hospital, the First Affiliated Hospital of Zhengzhou University from January 2014 to December 2021.The clinical characteristics, diagnosis and treatment process, and follow-up results were summarized, and the survival rate was calculated using the Kaplan-Meier method. Results Among the 12 patients, 10 were males, and 2 were females, with the age of onset of 2.8 (1.0-5.7) years.CCSK originated from the left kidney in 6 patients and from the right kidney in 6 patients.All the patients underwent complete resection of the tumor, including 2 patients in stage Ⅰ, 4 patients in stage Ⅱ, 3 patients in stage Ⅲ, and 3 patients in stage Ⅳ and 12 children received the corresponding chemotherapy regimen, 6 cases received radiotherapy.During the follow-up of 56 (19-94) months to December 31, 2023, 4 cases died of disease recurrence, and 8 cases survived without disease.The 5-year event-free survival rate was (62±11)%, and the 5-year overall survival rate was (72±9)%. Conclusions Pediatric CCSK is more common in male children.The combination of radical surgery and postoperative chemoradiotherapy offers a good prognosis in the early stage, while the mortality of patients due to recurrence and distant metastasis is high.
关键词
儿童/肾透明细胞肉瘤/治疗/预后
Key words
Child/Clear cell sarcoma of kidney/Treatment/Prognosis
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