肝脏间叶性错构瘤临床病理特征分析

Clinicopathological features of hepatic mesenchymal hamartoma

目的探讨肝脏间叶性错构瘤(MHL)的临床病理特征、免疫表型、诊断及鉴别诊断。方法回顾性分析2例MHL的临床病理资料和免疫表型,并复习相关文献。结果例1,男童,14个月,发现腹部膨隆2~3个月入院。例2,女童,14岁,无明显诱因腹部逐渐增大2个月余入院。影像学腹部CT:2例均显示腹腔巨大囊实性肿块,1例考虑良性肿瘤可能性大;另1例考虑恶性,未分化胚胎性肉瘤待排。病理学镜检:肿瘤主要由疏松结缔组织、扭曲分支状的胆管及不规则肝细胞岛组成。疏松结缔组织内含片状黏液样基质,其内散在梭形细胞、星形细胞。免疫表型:与文献报道一致,肿瘤内肝细胞表达CK(AE1/AE3)、Hepatocyte,胆管上皮细胞表达CK(AE1/AE3)、CK7、CKl9,血管CD31、CD34阳性,间质梭形细胞Vimentin阳性,Desmin、SMA灶阳,GPC3阴性,Ki-67阳性指数<1%。2例均手术完全切除,随访无复发或恶变。结论MHL是一种少见的良性肝脏肿瘤,偶有恶变可能,需及时手术,术后长期随访。

Objective To investigate the clinicopathological features and immunophenotype of mesenchymal hamartoma of liver(MHL)with emphasis on diagnosis and differential diagnosis.Methods Two cases of MHL were retrospectively analyzed about the clinicopathological and immunophenotypic profiles with the review of related literatures.Results One patient was 14-months-old boy,who was found abdominal distention for about 2-3 months.Another was a 14-year-old girl,and she came under observation gradual enlargement of his abdomen without obvious inducement.Computed tomography(CT)of their abdomen both revealed a large,intra-peritoneal lesion,with thick wall,multiple cysts of variable size and solid septa.One of them was considered benign probably,but the other case was likely to be malignant.Pathologically,the major components of MHL were loose connective tissue,malformed bile ducts and small groups of hepatocytes in variable proportions,and loose connective tissue often contained myxoid matrix scattered with spindle cells and astrocytes.By immunohistochemistry,the cord of hepatocytes within the tumor expressed CK(AE1/AE3);Hepatocyte and biliary epithelial cell expressed CK(AE1/AE3),CK7,CKl9;meanwhile CD31 and CD34 were positive in blood vessel.Interstitial spindle cells were variable reactivity for vimentin,desmin and SMA,but negative for S-100 and GPC3.The proliferation index of Ki-67 was under 1%.Both of the lesions were surgically resected,and there were no signs of recurrence or malignant transformation after long follow-up.Conclusion MHL represents a rare benign hepatic tumor,with a low potential of malignant transformation.SO early surgical treatment and long-term follow-up are needed.