1例儿童肝罕见组织学形态上皮样炎性肌纤维母细胞肉瘤的临床病理分析

Clinicopathologic analysis of a case of rare histopathologically morphologic epithelioid inflammatory myofibroblastic sarcoma in the liver of children

采用光镜、免疫组织化学及荧光原位杂交等方法分析1例以微囊性、腺泡样及实性结构为主的儿童肝上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma,EIMS)临床病理特征,结合文献复习,做出相应鉴别。患者为3岁女童,肝巨大占位。术后病理检查:镜下见肿瘤与肝组织界限较清,肿瘤主体部分呈疏密不均的微囊样或肺泡样结构,囊壁被覆多角形或短梭形上皮样细胞,囊腔内大量淋巴细胞、浆细胞、组织细胞浸润;局部上皮样细胞呈腺泡样至实性片状。免疫组织化学:上皮样细胞CK18、Vim、Desmin强阳性,ALK阴性,Ki-67指数约3%。综合分析后诊断为EIMS,术后9个月随访,患儿状况良好,无肿瘤复发等异常。本例特殊组织学形态EIMS,未见有文献报道描述,诊断主要依据形态学、免疫组织化学及分子检测,临床病理上需和多个肿瘤鉴别。治疗以手术扩大切除为主,需密切随访,进一步了解其生物学行为及预后。

The clinicopathological features of 1 case of pediatric liver epithelioid inflammatory myofibroblastic sarcoma(EIMS)with microcystic,acinar and solid structures were analyzed by light microscopy,immunohistochemistry and fluorescence in situ hybridization,and the corresponding differentiation was made based on literature review.The patient was a 3-year-old girl with a large liver space.Postoperative pathologic microscopy showed that the tumor was clearly bounded from the liver tissue,and the main body of the tumor presented an uneven microcystic or alveolar-like structure,with the cyst wall covered with polygonal or short-spindle epithelioid cells,and a large number of lymphocytes,plasma cells and tissue cells infiltrated in the cyst cavity and stroma.Local epithelioid cells were acinar to solid sheets.Immunohistochemistry:epithelial tumor cells CK18,Vim,and Desmin were strongly positive,and ALK was negative,with the Ki-67 index of about 3%.After comprehensive analysis,the patient was diagnosed as epithelioid inflammatory myofibroblastic tumor.The patient was followed up 9 months after the operation and was in good condition without tumor recurrence or other abnormalities.The special histological morphology of this case is EIMS,which has not been described in the literature,and the diagnosis is mainly based on morphological,immunohistochemical and molecular detection.Clinically and pathologically,multiple tumors need to be identified.The main treatment is surgical resection,and close follow-up is needed to understand its biological behavior and prognosis.