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中华诊断学电子杂志 ›› 2022, Vol. 10 ›› Issue (02) : 137 -142. doi: 10.3877/cma.j.issn.2095-655X.2022.02.014

病例诊断思维

β-catenin突变激活型肝细胞腺瘤的诊断学特征并文献复习
宁春景1, 王双龙2, 吕镔2,()   
  1. 1. 272013 济宁医学院临床医学院;272002 济宁市第一人民医院超声医学科
    2. 272002 济宁市第一人民医院超声医学科
  • 收稿日期:2021-07-26 出版日期:2022-04-26
  • 通信作者: 吕镔
  • 基金资助:
    济宁市新旧动能转换计划项目(2017SMNS011)

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

Chunjing Ning1, Shuanglong Wang2, Bin Lyu2,()   

  1. 1. College of Clinical Medicine, Jining Medical University, Jining 272013, China; Department of Ultrasound, Jining NO.1 People′s Hospital, Jining 272002, China
    2. Department of Ultrasound, Jining NO.1 People′s Hospital, Jining 272002, China
  • Received:2021-07-26 Published:2022-04-26
  • Corresponding author: Bin Lyu
引用本文:

宁春景, 王双龙, 吕镔. β-catenin突变激活型肝细胞腺瘤的诊断学特征并文献复习[J/OL]. 中华诊断学电子杂志, 2022, 10(02): 137-142.

Chunjing Ning, Shuanglong Wang, Bin Lyu. Diagnostic features of β-catenin mutated activated hepatocellular adenoma and literature review[J/OL]. Chinese Journal of Diagnostics(Electronic Edition), 2022, 10(02): 137-142.

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 of β-catenin mutated activated hepatocellular adenoma (β-HCA).

Methods

The clinical and imageology data of a case of β-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.

Results

The 31-year-old female patient, without any clinical symptoms, was admitted to hospital after physical examination with focal liver lesions. The ultrasonic manifestations of β-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 + ), and β-HCA was confirmed.

Conclusions

β-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.

图1 β-联蛋白突变激活型肝细胞腺瘤患者病灶二维超声图像注:肝右叶病灶为稍高回声,边界清,周缘回声减低,内回声不均匀,中央区可见一条状强回声(箭头所示)
图2 β-联蛋白突变激活型肝细胞腺瘤患者肝右叶超声造影图像注:a图示动脉期(8 s)肝右叶病灶呈不均匀高增强,病灶周边动脉相早期见肿瘤的供养动脉显影(箭头所示),病灶内出血坏死区无增强(三角形所示);b图示门脉期(35 s)肝右叶病灶廓清,病灶内出血坏死区无增强(三角形所示);c图示延迟期(3 min)肝右叶病灶呈低增强,病灶内出血坏死区无增强(三角形所示)
图3 β-联蛋白突变激活型肝细胞腺瘤患者腹部增强CT检查图像注:肝右叶病灶增强扫描密度不均。a图示动脉期肝右叶病灶CT值为69 Hu,病灶呈环形向心性强化;b图示门脉期造影剂向病灶内部填充,肝右叶病灶CT值升高为81 Hu;c图示造影剂逐渐廓清,延迟期肝右叶病灶CT值为74 Hu(箭头所示)
图4 β-联蛋白突变激活型肝细胞腺瘤患者腹部MRI检查图像注:a图示动脉早期肝右叶病灶呈环形强化;b图及c图示门脉期及延迟期肝右叶病灶不均质强化;d图示肝胆特异期肝右叶病灶大部分未见摄取,呈低信号,局部边缘摄取(箭头所示)
图5 β-catenin突变激活型肝细胞腺瘤患者肝占位镜下及免疫组织化学染色图像注:a图示肿瘤细胞,细胞较丰富(HE ×200);b图示β-catenin肿瘤细胞膜阳性(EnVision法 ×200);c图示GS肿瘤细胞质弥漫强阳性(EnVision法 ×200)(箭头所示);β-catenin为β-联蛋白;GS为谷氨酰胺合成酶;EnVision法为多聚螯合物酶法
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