儿童肾透明细胞肉瘤的临床分析
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摘要
目的:探讨儿童肾透明细胞肉瘤的临床特点及肾脏肿瘤治疗方案(WT-2009方案)治疗该病的疗效和安全性。方法:收集天津医科大学肿瘤医院2009年1月至2014年12月收治的肾透明细胞肉瘤患儿病例12例,因临床数据不完整等原因剔除3例,共计9例资料,对其临床表现和治疗过程以及随访结果进行回顾性分析。结果:经综合治疗后9例患儿均完全缓解(100%),随访满3年的6例患儿,3年总生存率100%,3年无事件生存率83.3%,未见明显治疗相关不良反应。结论:肾透明细胞肉瘤发病率低,误诊率高,WT-2009治疗方案近期疗效好,高危患者的治疗方案有待完善,远期治疗效果需进一步随访。
Objective: To summarize the clinical features of clear cell sarcoma of the kidney(CCSK) in children and to evaluate the efficacy 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 patients.Results: Nine cases of CCSK were recruited,and another three cases were excluded for the analysis because of incomplete clinical data.All patients achieved complete remission when the treatment was finished.The estimated 3-year overall survival rate was100%,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 protocol 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.
Objective: To summarize the clinical features of clear cell sarcoma of the kidney(CCSK) in children and to evaluate the efficacy 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 patients.Results: Nine cases of CCSK were recruited,and another three cases were excluded for the analysis because of incomplete clinical data.All patients achieved complete remission when the treatment was finished.The estimated 3-year overall survival rate was100%,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 protocol 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.
引文
[1]Gooskens SL,Furtw?ngler R,Vujanic GM,et al.Clear cell sarcoma of the kidney:a review[J].Eur J Cancer,2012,48(14):2219-2226.
[2]Oue T,Fukuzawa M,Okita H,et al.Outcome of pediatric renal tumor treated using the Japan Wilms Tumor Study-1(JWi TS-1)protocol:a report from the JWi TS group[J].Pediatr Surg Int,2009,25(11):923-929.
[3]Chinese Children's Cancer Group,Chinese Anti-Cancer Association.Long-term follow-up report of multicenter clinical study on childhood renal tumor[J].Chin J Pediatr,2016,54(11):808-813.[中国抗癌协会儿科专业委员会.儿童肾肿瘤多中心协作方案诊治随访报告[J].中华儿科杂志,2016,54(11):808-813.]
[4]Therasse P,Arbuck SG,Eisenhauer EA,et al.New guidelines to evaluate the response to treatment in solid tumors[J].Breast Cancer,2004,92(1):881-886.
[5]Huang CC,Cutcliffe C,Coffin C,et al.Classification of malignant pediatric renal tumors by gene expression[J].Pediatri Blood Cancer,2006,46(7):728-738.
[6]Brok J,Treger TD,Gooskens SL,et al.Biology and treatment of renal tumours in childhood[J].Eur J Cancer,2016,68:179-195.
[7]Uenoyokohata H,Okita H,Nakasato K,et al.Consistent in-frame internal tandem duplications of BCOR characterize clear cell sarcoma of the kidney[J].Nature Genetics,2015,47(8):861.
[8]Furtw?ngler R,Gooskens SL.Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology(SIOP)93-01and SIOP 2001 protocols:a report of the SIOP Renal Tumour Study Group[J].Eur J Cancer,2013,49(16):3497-3506.
[9]Yu LW,Li HM,Ling BW,et al.Imaging findings of clear cell sarcoma of the kidney in children[J].J Clin Radiol,2016,35(3):438-440.[虞崚崴,李惠民,凃备武,等.儿童肾透明细胞肉瘤的CT与MRI表现[J].临床放射学杂志,2016,35(3):438-440.]
[10]Hadley GP,Sheik-Gafoor MH.Clear cell sarcoma of the kidney in children:experience in a developing country[J].Pediatric Surg Int,2010,26(4):345-348.
[11]He LJ,Fu LB,Wang L,et al.A clinicopathological study of clear cell sarcoma of the kidney[J].Chin J Pathol,2001,30(6):422-425.[何乐健,伏利兵,王琳,等.肾透明细胞肉瘤的临床病理学研究[J].中华病理学杂志,2001,30(6):422-425.]
[12]Wang CB,Chen GS,Li J,et al.Clinicopathologic analysis and literature review of clear cell sarcoma of the kidney in children[J].J Mod Oncol,2014,(10):2417-2420.[王春宝,陈广生,李娟,等.儿童肾透明细胞肉瘤临床病理分析及文献复习[J].现代肿瘤医学,2014,(10):2417-2420.]
[13]Sheng Q,Liu TT,He KJ,et al.Multidisciplinary diagnosis,treatment,and follow-up of children with clear cell sarcoma of kidney[J].J Shanghai Jiaotong Univ Med Sci,2017,37(2):225-229.[盛琦,刘婷婷,何珂骏,等.儿童肾透明细胞肉瘤的多学科诊治与随访[J].上海交通大学学报(医学版),2017,37(2):225-229.]
[14]Argani P,Perlman EN,Browning N,et al.Clear cell sarcoma of the kidney:a review of 351 cases from the National Wilms Tumor Study Group Pathology Center[J].Am J of Surgical Pathology,2000,24(1):4.
[15]Weaver J,Ho T,Lang A,et al.Bladder Recurrence of Clear Cell Sarcoma of the Kidney 7 Years after Initial Presentation[J].Urology,2017,106:193-195.
[2]Oue T,Fukuzawa M,Okita H,et al.Outcome of pediatric renal tumor treated using the Japan Wilms Tumor Study-1(JWi TS-1)protocol:a report from the JWi TS group[J].Pediatr Surg Int,2009,25(11):923-929.
[3]Chinese Children's Cancer Group,Chinese Anti-Cancer Association.Long-term follow-up report of multicenter clinical study on childhood renal tumor[J].Chin J Pediatr,2016,54(11):808-813.[中国抗癌协会儿科专业委员会.儿童肾肿瘤多中心协作方案诊治随访报告[J].中华儿科杂志,2016,54(11):808-813.]
[4]Therasse P,Arbuck SG,Eisenhauer EA,et al.New guidelines to evaluate the response to treatment in solid tumors[J].Breast Cancer,2004,92(1):881-886.
[5]Huang CC,Cutcliffe C,Coffin C,et al.Classification of malignant pediatric renal tumors by gene expression[J].Pediatri Blood Cancer,2006,46(7):728-738.
[6]Brok J,Treger TD,Gooskens SL,et al.Biology and treatment of renal tumours in childhood[J].Eur J Cancer,2016,68:179-195.
[7]Uenoyokohata H,Okita H,Nakasato K,et al.Consistent in-frame internal tandem duplications of BCOR characterize clear cell sarcoma of the kidney[J].Nature Genetics,2015,47(8):861.
[8]Furtw?ngler R,Gooskens SL.Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology(SIOP)93-01and SIOP 2001 protocols:a report of the SIOP Renal Tumour Study Group[J].Eur J Cancer,2013,49(16):3497-3506.
[9]Yu LW,Li HM,Ling BW,et al.Imaging findings of clear cell sarcoma of the kidney in children[J].J Clin Radiol,2016,35(3):438-440.[虞崚崴,李惠民,凃备武,等.儿童肾透明细胞肉瘤的CT与MRI表现[J].临床放射学杂志,2016,35(3):438-440.]
[10]Hadley GP,Sheik-Gafoor MH.Clear cell sarcoma of the kidney in children:experience in a developing country[J].Pediatric Surg Int,2010,26(4):345-348.
[11]He LJ,Fu LB,Wang L,et al.A clinicopathological study of clear cell sarcoma of the kidney[J].Chin J Pathol,2001,30(6):422-425.[何乐健,伏利兵,王琳,等.肾透明细胞肉瘤的临床病理学研究[J].中华病理学杂志,2001,30(6):422-425.]
[12]Wang CB,Chen GS,Li J,et al.Clinicopathologic analysis and literature review of clear cell sarcoma of the kidney in children[J].J Mod Oncol,2014,(10):2417-2420.[王春宝,陈广生,李娟,等.儿童肾透明细胞肉瘤临床病理分析及文献复习[J].现代肿瘤医学,2014,(10):2417-2420.]
[13]Sheng Q,Liu TT,He KJ,et al.Multidisciplinary diagnosis,treatment,and follow-up of children with clear cell sarcoma of kidney[J].J Shanghai Jiaotong Univ Med Sci,2017,37(2):225-229.[盛琦,刘婷婷,何珂骏,等.儿童肾透明细胞肉瘤的多学科诊治与随访[J].上海交通大学学报(医学版),2017,37(2):225-229.]
[14]Argani P,Perlman EN,Browning N,et al.Clear cell sarcoma of the kidney:a review of 351 cases from the National Wilms Tumor Study Group Pathology Center[J].Am J of Surgical Pathology,2000,24(1):4.
[15]Weaver J,Ho T,Lang A,et al.Bladder Recurrence of Clear Cell Sarcoma of the Kidney 7 Years after Initial Presentation[J].Urology,2017,106:193-195.