小儿肝母细胞瘤的多学科联合诊疗回顾性分析

Retrospective analysis of multi-discipline combination treatment of hepatoblastoma in children

目的探讨多学科联合诊疗在小儿肝母细胞瘤诊治中的价值。方法回顾性分析2013年1月至2019年4月微创外科收治13例经多学科联合诊疗的小儿肝母细胞瘤的临床资料。通过回顾患儿病史、影像学资料、手术记录、治疗前病变分期(PRETEXT)、病理学诊断、术前及术后化疗方案,通过门诊、电话对患儿生存情况进行随访。结果13例患儿经多学科联合讨论确定术前PRETEXT,其中Ⅰ期1例(7.7%),Ⅱ期2例(15.4%),Ⅲ期7例(53.8%),Ⅳ期3例(23.1%);术后病理结果:胎儿型3例(23.1%),胚胎型4例(30.8%),巨小梁型2例(15.4%),间变型(小细胞未分化型)1例(7.7%),混合型3例(23.1%)。1例I期患儿直接行肝叶切除术,11例患儿术前行C5V方案化疗3~5个疗程后行肝脏肿瘤切除术,术后给予同样方案化疗4~7个疗程。随防时间8~73个月,中位随防时间33个月,共生存10例,死亡3例,生存率76.9%。PRETEXTⅢ期患儿生存率低于PRETEXTⅣ期(P<0.05);高危组(PRETEXTⅣ期)患儿生存率低于标准组(PRETEXTⅠ、Ⅱ、Ⅲ期)(P<0.05);间变型患儿生存率低于非间变型(P<0.05);巨小梁型患儿生存率低于非巨小梁型(P<0.05)。结论手术联合化疗是小儿肝母细胞瘤治疗的关键,多学科联合诊治能保证整个疗程顺利进行,取得较好临床效果。

Objective To investigate the application value of multi-discipline combination treatment in diagnosis and treatment of hepatoblastoma in children.Methods The clinical data about 13 patients with hepatoblastoma who were treated by multi-discipline combination treatment in our hospital from January 2013 to April 2019 were retrospective analyzed.The observation indexes included medical history,imaging data,operation record,PRETEXT staging before surgery,pathological diagnosis,preoperative and postoperative chemotherapy protocols.Moreover the patients were followed up by outpatient examination and telephone interviews.Results All the 13 patients underwent multi-disciplinary discussion to decide preoperative PRETEXT,in which,there was 1 case of stageⅠ(7.7%,1/13),2 cases of stageⅡ(15.4%,2/13),7 cases of stageⅢ(53.8%,7/13)and 3 cases of stage IV(23.1%,3/13).The postoperative pathological results showed that there were 3 cases of fetal type(23.1%,3/13),4 cases of embryo type(30.8%,4/13),2 cases of giant trabecular type(15.4%,2/13),1 case of anaplasia(small cell undifferentiated type,7.7%,1/13)and 3 cases of mixed type(23.1%,3/13).The 1 patient with stage I was treated by hepatectomy,and 11 cases received C5V chemotherapy before surgery and liver tumor resection after 3~5 courses of chemotherapy,and the same chemotherapy protocol was performed for 4~7 treatment courses after operation.The patients were followed-up for 8~73 months,with the median follow-up time being 33 months.In addition 10 patients were survival and 3 cases died,with survival rate being 76.9%.The survival rate of patients at PRETEXT stageⅢwas significantly lower than that of patients at PRETEXT stageⅣ(P<0.05),and the survival rate in high-risk group(PRETEXTⅣ)was significantly lower than that in standard group(PRETEXTⅠ,Ⅱ,Ⅲ)(P<0.05),which in the patients with metaplasia was significantly lower than that of patients without metaplasia(P<0.05),and the survival rate of patients with giant trabecular type was significantly lower than that of patients with non-giant trabecular type(P<0.05).Conclusion The surgery combined with chemotherapy is the key to the treatment of hepatoblastoma in children.Multi-disciplinary combination diagnosis and treatment can ensure the smooth progress of the whole course of treatment and achieve satisfactory clinical results.