β-catenin突变激活型肝细胞腺瘤的诊断学特征并文献复习

Diagnostic features of β-catenin mutated activated hepatocellular adenoma and literature review

目的探讨β-catenin突变激活型肝细胞腺瘤(β-HCA)的诊断学特征。方法回顾性分析2020年2月6日济宁市第一人民医院超声医学科经穿刺活检病理证实的1例β-HCA患者的临床及影像检查资料,并文献复习。结果患者女性,31岁,无任何临床症状,体检发现肝占位入院;β-HCA超声表现为类圆形富血供的稍高回声团块,内回声不均匀;超声造影示动脉期病灶快速增强,门脉期和延迟期低增强。穿刺标本病理学检查示肝细胞腺瘤,伴紫癜样出血;免疫组织化学染色:细胞角蛋白8(CK8)(+++),白细胞分化抗原34(CD34)(毛细血管+),CD10(局灶+),热休克蛋白70(HSP70)(局灶+),谷氨酰胺合成酶(GS)(+++),β-联蛋白(β-catenin)(细胞膜+),确诊为β-HCA。结论β-HCA临床表现无特异性,超声造影可表现为动脉期快速增强,门脉期可快速廓清或等增强,延迟期低增强或等增强,最终诊断需依赖组织病理学和免疫组织化学分析。

Objective To investigate the diagnostic characteristics ofβ-catenin mutated activated hepatocellular adenoma(β-HCA).MethodsThe clinical and imageology data of a case ofβ-HCA pathologically confirmed by puncture biopsy on February 6,2020 in the Ultrasound Department of Jining NO.1 People′s Hospital were retrospectively analyzed,and the literatures were reviewed.ResultsThe 31-year-old female patient,without any clinical symptoms,was admitted to hospital after physical examination with focal liver lesions.The ultrasonic manifestations ofβ-HCA were slightly circular higher echo with rich blood supply and uneven internal echo.Contrast-enhanced ultrasound showed rapid enhancement in arterial phase and low enhancement in portal and delayed phases.Histopathological examination revealed hepatocellular adenoma with purpuric hemorrhage.Immunohistochemistry:cytokeratin 8(CK8)(+++),cluster of differentiation antigen 34(CD34)(capillary+),CD10(focal+),heat shock protein 70(HSP70)(focal+),glutamine synthase(GS)(+++),β-catenin(membrane+),andβ-HCA was confirmed.Conclusionsβ-HCA has no specific clinical manifestations.Contrast-enhanced ultrasound can show rapid enhancement in arterial phase,rapid clearance or equal enhancement in portal vein phase,low enhancement or equal enhancement in delayed phase,the final diagnosis depends on histopathological and immunohistochemical analysis.