小儿肝母细胞瘤78例临床病理分析

Clinicopathological analysis of 78 cases of children with hepatoblastoma

目的分析小儿肝母细胞瘤(HB)的组织学形态、免疫表型、诊断及鉴别诊断。方法回顾性分析78例HB患儿的临床病理资料、组织学形态特点、免疫组化及预后。结果78例HB患儿中,男46例,女32例,男:女=1.4∶1。年龄<5岁的患儿占总数的87.18%。55.13%病例临床表现为腹部包块。96.15%的患儿血肿瘤标记物AFP升高。肿瘤累及肝右叶例数占总数的71.79%;肝左叶例数占总数的11.54%;肝左右叶均累及例数占总数的16.67%。本研究中,83.33%患儿为单发肿瘤,16.67%患儿为多发肿瘤。组织学上,单纯胎儿型48例(61.54%),混合性胎儿型和胚胎型21例(26.92%),小细胞未分化型2例(2.56%),混合性上皮和间叶型7例(8.97%)。随访2~78个月,66例有效随访患儿总生存率为74.24%。结论HB多发生于5岁以下儿童,男孩多于女孩,临床多表现为腹部包块,多位于肝右叶,常为单发,血清AFP升高,且其组织学分型及临床分期与肿瘤的预后密切相关。

Objective To analyze the histomorphology,immunophenotype,diagnosis and differential diagnosis of hepatoblastoma(HB)in children.Methods The clinicopathological data,histological features,immunohistochemistry and prognosis of 78 cases of HB in children were retrospectively analyzed.Results Among the 78 cases of HB,there were 46 boys and 32 girls,and the proportion of boy and girl was 1.4 and 1.Children with HB under 5 years old accounted for 87.18%of the total.The clinical manifestation of 55.13%cases was abdominal mass.Blood tumor marker AFP elevated in 96.15%of children.Among the children,the right lobe tumors of liver was 71.79%of the total,the left lobe tumors of liver was 11.54%,and the left and right lobe tumors of liver were 16.67%.In this study,83.33%of the children with HB were single tumors and 16.67%were multiple tumors.Histologically,48 cases were pure fetal type(61.54%),21 cases were mixed fetal type and embryonal type(26.92%),2 cases were small undifferentiated type(2.56%)and 7 cases were mixed epithelial mesenchymal type(8.97%).After 2-78 months'follow-up,it showed that the total survival rate of 66 children was 74.24%.Conclusion HB mostly occurs in children under 5 years old.Boys have more incidence than females.The clinical manifestation of the HB is abdominal mass.Most of the tumors are located in the right lobe of the liver,often single.The serum AFP of the children is elevated.The histological classification and clinical stage are closely related to the prognosis of the tumors.