儿童肝母细胞瘤临床病理分析

Clinicopathological analysis of Hepatoblastoma in children

目的探讨儿童肝母细胞瘤(hepatoblastoma,HB)的临床病理特征、免疫表型及鉴别诊断。方法回顾性分析我院7例HB的临床资料、组织病理学特征,免疫表型及预后,并结合相关文献进行讨论。结果本组男女之比为4:3,年龄为11个月-4岁,平均年龄2岁。7例病例5例经手术切除,2例经粗针穿刺活检确诊。术后随访6-30个月,6例获得随访资料,手术或穿刺活检确诊后化疗现均存活。病理形态包括单纯胎儿型1例,混合胎儿和胚胎型3例,混合上皮和间叶型3例。免疫表型:AFP、Hepatocyte、Glypican-3、β-catenin呈不同程度阳性、CD34血管内皮细胞阳性,NSE、S100、Syn、CgA、CD99、Des,EMA,LCA均阴性,Ki-67增殖指数为40%-90%。结论HB是儿童最常见的肝脏来源的恶性肿瘤,多发生于5岁以下儿童,该病起病隐匿,早期多无症状,部分患儿发现时已有远处转移。临床多表现为腹部包块,血清AFP升高是一个重要的参考指标,最终确诊还需依赖病理诊断。

Objective To investigate the Clinicopathologic features,immunophenotype and differential diagnosis of Hepatoblastoma(HB)in children.Methods The clinical data,histopathology,immunophenotype and prognosis of 7 patients with HB in our hospital were analyzed retrospectively.Results The ratio of male to female was 4:3,the age ranged from 11 months to 4 years,the average age was 2 years.7 cases(5 cases)were resected and 2 cases were diagnosed by needle biopsy.Six patients were followed up for 6 to 30 months,and all survived after surgery or biopsy.The pathological features included simple fetal type in 1 case,mixed fetal and embryonic type in 3 cases,mixed epithelial and Mesenchymal type in 3 cases.Immunophenotype:AFP,Hepatocyte,Glypican-3,β-Catenin were positive in different degree and CD34 Endothelium,NSE,S100,Syn,CgA,CD99,Des,EMA,LCA were negative,Ki-67 proliferation index was 40%-90%.Conclusion HB is the most common liver-derived malignant tumor in children,most of which occurs in children under 5 years of age.The clinical manifestations were mostly abdominal mass.The increase of serum AFP was an important reference index.The final diagnosis still depended on pathological diagnosis.