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目的探讨儿童肝母细胞瘤的多学科协作诊断及治疗。方法回顾分析1998年8月-2006年1月上海儿童医学中心小儿外科收治的16例肝母细胞瘤患儿(男女各8例;发病年龄3个月～11岁,平均2岁)的治疗及随访情况。着重分析肝母细胞瘤多学科协作治疗、术前评估及手术要点。结果发病部位在肝右叶、肝左叶各8例。临床表现为腹部肿块15例,腹膜炎体征1例。采用国际儿童肿瘤协会(SIOP)Pretext分期法分期,Ⅱ、Ⅲ期各7例(各占43.7%,7/16例),Ⅳ期2例(占12.5%,2/16例)。Ⅱ期7例中,6例行肿瘤完整切除,1例因肿瘤破裂急诊行剖腹探查术,术后行ICE方案化疗。Ⅲ期6例开放活检明确病理诊断,术前化疗2、3个疗程,肿瘤缩小后手术完整切除,1例放弃治疗。Ⅳ期1例行肝移植,1例放弃治疗。Ⅱ期随访7例,6例存活,1例死亡,3a无瘤生存率为100%(4/4例)。Ⅲ期随访7例,5例存活,2例死亡,3a无瘤生存率为75%(3/4例)。Ⅳ期随访2例,肝移植后4个月因肝衰竭死亡1例,放弃治疗死亡1例。结论手术完整切除在儿童肝母细胞瘤治疗中起举足轻重的作用,辅助化疗能够提高肿瘤的完整切除率,多学科协作治疗能够保证整个疗程的顺利进行,取得良好的临床疗效。
Objective To investigate the multidisciplinary diagnosis and treatment of hepatoblastoma in children. Methods The clinical data of 16 patients with hepatoblastoma (8 males and 8 females; age from 3 months to 11 years, mean 2 years) admitted to pediatric surgery department of Shanghai Children’s Medical Center from August 1998 to January 2006 were analyzed retrospectively. Follow-up situation. Focus on analysis of hepatoblastoma multidisciplinary collaborative treatment, preoperative evaluation and surgical points. Results of the disease in the right lobe, left lobe of the liver in 8 cases. Clinical manifestations of abdominal mass in 15 cases, 1 case of peritonitis signs. Seven cases (43.7% and 7/16 cases) in stage Ⅱ and Ⅲ were treated by Pretext staging method of SIOP, and 2 cases (12.5% and 2/16 cases) in stage Ⅳ. Among the 7 cases of stage Ⅱ, 6 cases underwent complete tumor resection, 1 case underwent exploratory laparotomy due to tumor rupture and ICE regimen was performed after operation. 6 cases of open biopsy confirmed the pathological diagnosis, preoperative chemotherapy 2,3 courses, complete resection of the tumor after the reduction surgery, 1 case to give up treatment. One patient underwent liver transplantation in stage Ⅳ and one patient gave up treatment. Seven patients were followed up in phase Ⅱ, 6 survived, 1 died, and the 3-year survival rate was 100% (4/4 patients). Three patients were followed up in 7 cases, 5 survived, 2 died and the 3-year tumor-free survival rate was 75% (3/4 cases). Two cases were followed up in stage Ⅳ. One died of liver failure in 4 months after liver transplantation, and one case was given up for treatment. Conclusion Complete resection of the tumor plays an important role in the treatment of hepatoblastoma in children. Adjuvant chemotherapy can improve the complete resection rate of the tumor. Multidisciplinary collaborative therapy can ensure the smooth progress of the whole course of treatment and achieve good clinical efficacy.