累及第二、三肝门巨大肝母细胞瘤手术切除41例

Surgical resection of huge hepatoblastoma involving the second and third hepatic hilums

目的：探讨累及第二、三肝门的巨大肝母细胞瘤手术切除的可行性及手术方法，以期扩大手术适应证，提高小儿肝母细胞瘤的治疗效果。方法我们对41例累及第二、三肝门的巨大肝母细胞瘤施行肿瘤切除手术，患儿年龄3个月至14岁，平均年龄3.8岁；其中男25例，女16例；肿瘤直径8.5～21.6 cm，平均13.5 cm；均行手术前化疗，化疗3至7个疗程不等，直至肿瘤不再缩小。采用Kap-lan-Meier生存分析计算本组患儿生存率。结果41例均顺利实施肝脏肿瘤切除术，手术时间125～350 min（平均220 min），术中出血40～500 mL，8例未输血。第一肝门阻断时间15～35 min （平均22 min），其中阻断1次者12例，2次者23例，2次以上者6例，每次阻断时间一般不超过20 min。手术切除方式包括V-Ⅷ段切除8例；Ⅳ-Ⅷ段切除9例；Ⅰ-Ⅳ段切除4例；Ⅰ、Ⅳ、Ⅷ段切除9例；Ⅰ、Ⅳ、Ⅴ、Ⅷ段切除6例；Ⅰ段＋Ⅴ-Ⅷ段切除2例；Ⅰ段＋Ⅳ-Ⅷ段切除3例；合并下腔静脉瘤栓7例。41例患儿1年、3年、5年无瘤生存率分别为92.7％、72.8％和33.5％。其中最长无瘤生存期为90个月。41例术后均经规范化疗，化疗后定期门诊复查，其中20例（48.8％）复发，继续化疗。5例出现肺转移病例中，2例经术后化疗转移灶消失，3例于术后1年内死亡。结论累及第二、三肝门的巨大肝母细胞瘤患儿手术切除虽然具有一定挑战性，但在详细、准确的术前评估，及对肝脏解剖的熟练掌握下，肿瘤切除仍具有一定的可行性和安全性。完整切除肿瘤可有效提高肝母细胞瘤的长期生存率。

Objetive To investigate the feasibility and surgical procedure for huge hepatoblastoma invol-ving the second and third hepatic hilums. In order to expand the indications for hepatectomy,improve treatment of children with hepatoblastoma. Methods 41 patients with huge hepatoblastoma involving the second and third hepatic hilums were evaluated.The patients’age ranged from 3 month to 1 4 years with a mean of 3.8 years.The tumors’diameter ranged from 8.5 ~21.6 cm with a mean of 1 3.5 cm. Kaplan-Meier survival analy-sis was applied to evaluate the survival rates of patients with hepatoblastoma. Results Of the 41 patients who underwent hepatectomy,there was no operative mortality or morbidity.The mean duration of the operations was 220 minutes and the mean blood loss was 1 1 5 mL.The mean time of intermittent portal triad clamping was 22 min. Of the 41 patients,the 1-,3-,5-year even-free survival rate were 92.7%,72.8% and 33.5%,re-spectively. All patients underwent postoperatively regular chemotherapy,20 patients（48.8%）found tumor re-currence after hepatectomy. 5 patients suffered tumor metastases in lung and 3 of them died from that in one year. Conclusion Resecting huge hepatoblastoma involving the main hepatic veins and the retrohepatic vena cava is feasible and safe with accurate preoperative assessment and skillful mastery of the anatomy of the liver.Complete resection of this kind of tumors would effectively improve the long-term survival of hepatoblastoma.