小儿肝脏间叶性错构瘤12例诊治分析

Diagnosis and treatment of hepatic mesenchymal hamartoma in children

目的回顾性分析12例小儿肝脏间叶性错构瘤(MHL)的临床资料,以提高该病诊断与治疗水平。方法收集1996年1月至2009年12月小儿MHL12例,年龄1～72个月,临床表现主要为腹部包块、腹胀。8例甲胎蛋白(AFP)增高(48.2～9187.2μg/L)。4例患儿术前行超声引导下肿物穿刺活检术,仅有1例考虑为MHL,余3例未获明确诊断。术前有2例患儿因疑为肝母细胞瘤而分别行介入栓塞和化疗。3例行右半肝切除术,3例行肝左外叶切除术,2例行不规则右半肝切除术,2例行肝右三叶切除术,1例行左半肝切除术,1例行肝部分不规则切除术。结果所有患儿术后均恢复顺利,无并发症发生。术后随访7个月～14年,除1例合并胆道闭锁患儿在1岁时死亡、1例患儿带瘤存活外,余10例患儿均状况良好,无肿瘤复发,复查AFP均正常。结论小儿肝脏间叶性错构瘤是一种少见的肝脏良性肿瘤,结合术前AFP、B超、CT和肿物穿刺病理检查有助于诊断。鉴别困难者宜行剖腹探查,以便明确诊断。手术完整切除后预后良好。

Objective Clinical data of12 cases of hepatic mesenchymal hamartoma in children were reviewed in order to raise the level of diagnosis and treatment.Methods Twelve children with hepatic mesenchymal hamartoma whose age range from 4 to 147 months were treated in our hospital between January 1996 and December 2009.Abdominal mass and distension were the chief complaints.The AFP levels in 8 cases elevated to 48.2-9187.2 μg/L.Four tumor biopsys were performed preoperatively under B ultrasonography, only 1 patient got the diagnosis of mesenchymal hamartoma.2 patients received embolization and chemotherapy before operations were suspected of hepatoblastoma.All patients underwent operations including right hemihepatectomy in 3 cases, left lateral hepatic lobectomy in 3 cases, irregular right hemihepatectomy in 2 cases, right trilobectomy in 2 cases, left hemihepatectomy in 1 case, irregular hepatic partial resection in 1 case.Results All patients recovered uneventfully.All patients were followed up from 7 months to 14 years, 1 died at 1 year old because of biliary atresia, 1 with tumor survival, the other 10 patients get tumor free survival with normal AFP level.Conclusions Mesenchymal hamartoma is a rare benign tumor arise from liver in children.The diagnosis of it depend on combination of preoperative AFP level,ultrasound,CT and needle biopsy.Exploratory laparotomy is necessary if the diagnosis is difficult.The prognosis is good if the tumor is removed completely removed.