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多灶性肝母细胞瘤手术切除的单中心研究OA北大核心CSTPCD

Hepatectomy for multifocal hepatoblastoma:a single-center report

中文摘要英文摘要

目的 探讨多灶性肝母细胞瘤(hepatoblastoma,HB)手术切除治疗的可行性及有效性.方法 本研究为回顾性研究,以2014年4月至2022年12月首都医科大学附属北京儿童医院肿瘤外科收治的21例多灶性HB患儿为研究对象,收集患儿临床特征、手术方式及远期预后等资料,并与同期收治的58例中高危单灶性HB患儿进行疗效对比.结果 21例多灶性HB患儿中,11例为2个瘤灶,4例为3个瘤灶,1例为5个瘤灶,5例肝内瘤灶超过5个且无法明确计数.21例均接受肿瘤切除手术,其中9例行解剖性肝切除术,11例行不规则肝切除或瘤灶剜除术,1例行联合肝脏离断及门静脉结扎的分次肝切除术(associating liver partition and portal vein ligation for staged hepatectomy,ALPPS).20 例为 R0切除,1例为R1切除,术后无一例发生严重并发症.本组多灶性HB患儿接受手术联合化疗的多学科综合治疗后,中位随访时间57个月,18例患儿无瘤生存,3例死亡.本组多灶性HB患儿3年无事件生存率(event-free survival,EFS)为 57.58%,3 年总生存率(overall survival,OS)为 82.00%,局部进展累积发生率(cumulative incidence of local progression,CILP)为 38.00%.同期收治的单灶性中高危 HB 患儿上述指标分别为91.84%、74.96%、20.04%.两组数据对比,差异无统计学意义(P>0.05).结论 多灶性HB经规范外科手术切除辅助术前、术后化疗,可以获得较为满意的预后.

Objective To explore the feasibility and effectiveness of hepatectomy for multifocal hepato-blastoma(HB).Methods A retrospective analysis was conducted for 21 hospital cases of multifocal HB pa-tients from April 2014 to December 2022.Clinical characteristics,surgical procedures and long-term outcomes were collected and compared with 58 cases of unifocal hepatoblastoma patients with intermediate/high-risk group during the same period.Results Among 21 children with multifocal HB,there were 2 tumor foci(n=1 1),3 tumor foci(n=4)and 5 tumor foci(n=1).Five cases had more than five intrahepatic tumor foci and a definite count was impossible.Tumor resection was anatomical(n=9),irregular(n=11)and combined liver dissection and portal vein ligation for staged hepatectomy(ALPPS,n=1).R0 resection(n=20)and R1 re-section(n=1)were performed without serious postoperative complications.After receiving multidisciplinary comprehensive treatment of surgery plus chemotherapy,median follow-up time for multifocal HB was 57 months.The outcomes were disease-free survival(n=18)and death(n=3).The 3-year event free survival(EFS),3-year overall survival(OS)and cumulative incidence of local progression(CILP)in children with multifocal HB were 57.58%,82.00%and 38.00%respectively.The above parameters of single focus intermittent/high-risk HB admitted during the same period were 91.84%,74.96%and 20.04%respectively.No significant sta-tistical difference existed between two datasets.Conclusions For multifocal HB,standardized surgical resec-tion plus perioperative chemotherapy may achieve satisfactory outcomes.It provides an effective treatment for multifocal HB children.

杨维;韩建宇;杨深;刘珊;任清华;王焕民;秦红;成海燕;常晓峰;朱志云;冯俊

首都医科大学附属北京儿童医院肿瘤外科,北京 100045

肝母细胞瘤外科手术治疗结果临床研究儿童

HepatoblastomaSurgical Procedures,OperativeTreatment OutcomeClinical StudyChild

《临床小儿外科杂志》 2024 (006)

543-549 / 7

国家自然科学基金(82293660/82293665) National Natural Science Foundation of China(82293660 & 82293665)

10.3760/cma.j.cn101785-202310006-007

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