多学科协作模式诊断治疗儿童肝母细胞瘤

Model of Multidisciplinary Team Treatment on Hepatoblastoma in Children

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 study the diagnosis and treatment of the model of multidisciplinary team treatment on hepatoblastoma in children. Methods Retrospective analysis of treatment and long term follow - up of 16 patients with hepatoblastoma. 8 cases were boys,8 cases were girls;aged 3 months to 11 years old,average age was 2 years old） during Aug. 1998 to Jan. 2006 in Shanghai Children＇s Medical Center. Special in multidisciplinary team treatment,preoperative evaluate and operation. Results The morbility locum of 8 cases were in right liver lobe ,8 cases were in left liver lobe. Fifteen cases of the clinical presentation were abdominal mass. 1 case was peritonitis. According to Interna tional Society of Pediatric Oncology （SIOP） Pretext staging system,43.7 % （7/16） cases were stage Ⅱ. 43.7 % （ 7/16 ） cases were stage m. 12.5% （2/16） cases were stage Ⅳ. In 7 cases of stage Ⅱ,6 cases were accepted tumor total resection. 1 case was accepted emergency surgery because tumor was fracture. Patients were given ICE protocal. In 7 cases of stage Ⅲ ,6 cases were able to undergo delayed tumor resection following open biopsy and subsequent treatment with chemotherapy 2 -3 courses. One case gave up treatment because its had poor response to chemotherapy. In 2 cases of stage Ⅳ, 1 case was received liver transplantation due to poor respond to chemotherapy. One case was not received any treatment. In 7 cases of stage Ⅱ ,6 cases were alive,1 case died,3 - year free survival rate was 100% （4/4）. In 7 cases of stage m .5 cased were alive,3 - year free survival rate was 75 % （3/4）. Two cases of stage Ⅳ all died. Conclusions Surgery play a very important role in the treatment of hepatoblastoma in children. Chemotherapy has improved the resectability of the tumor. Multidisciplinary team treatment is an effective model for diagnosis and treatment on childhood hepatoblastoma.