肝脏炎性假瘤样滤泡树突状细胞肿瘤的临床病理分析

Clinical Pathological Factors of Hepatic Inflammatory Pseudotumor-like Follicular Dendritic Cell Tumor

目的探讨肝脏炎性假瘤样滤泡树突状细胞肿瘤的临床病理学特征,为临床提供诊断及鉴别诊断的依据。方法对1例肝脏炎性假瘤样滤泡树突状肿瘤、7列肝脏炎症性肌纤维母细胞性肿瘤、1例肝脏原发性霍奇金淋巴瘤及1例肝脏原发性恶性纤维组织细胞瘤进行临床病理分析、免疫组织化学染色及EBV-encoded RNA(EBER)原位杂交检测。结果肝脏炎症性肌纤维母细胞性肿瘤、肝脏原发性霍奇金淋巴瘤和肝脏原发性恶性纤维组织细胞瘤与肝脏炎性假瘤样滤泡树突状细胞肿瘤临床症状、影像学表现及镜下表现比较相似,但肝脏炎性假瘤样滤泡树突状细胞肿瘤在临床上可以无症状或右上腹不适或疼痛、肝肿大、发热、体重减轻等。肿瘤呈实性,与周围组织间有明显界限。镜下肿瘤细胞为梭形、卵圆形,呈束状、席纹状排列,并散在分布于以淋巴细胞和浆细胞为主的炎症细胞背景中。梭形细胞核较小,常被扭转和不规则折叠,呈空泡状,但核仁明显。还可见到R-S样细胞,且免疫表型CD21、CD35阳性,EBER原位杂交阳性。结论肝脏炎性假瘤样滤泡树突状细胞肿瘤是罕见的肿瘤,其形态的复杂性、相似性,使免疫组织化学标志物和EBER原位杂交成为诊断这类肿瘤不可或缺的辅助手段。

Objective To investigate the clinicopathologic features of hepatic inflammatory pseudotumor-like follicular dendritic cell tumor（IPT-like FDCT）,and to conclue the diagnostic and differential diagnostic criteria for these morphologically similar tumors.Methods One case of hepatic IPT-like FDCT,seven cases of hepatic inflammatory myofibroblastic tumor（IMT）,one case of primary hepatic Hodgkin lymphoma（HL）and one case of primary hepatic malignant fibrous histiocytoma（MFH） were analyzed by routine histopathological,immunohistochemical staining and in situ hybridization for Epstein-Barr Virus（EBV）-encoded RNA（EBER）.Results The hepatic IMT,primary hepatic HL and primary hepatic MFH were mimicking hepatic IPT-like FDCT in many ascpects of clinicopathologic features.However,hepatic IPT-like FDCT had no any symptom or right upper quadrant pain,hepatomegaly,fever and weight loss.The tumor is solitary and well demarcated from surrounding parechyma.Histologically,Tumor cells were composed of oval-to-spindle cells arranged in diffuse,vague storiform,fascicular and spindle cells were dispersed in a background of abundant inflammatory cells,predominantly lymphocytes and plasma cells.The cells possessed enlarged,sometimes twisted or irregularly folded,nuclei that contained vesicular chromatin,and small but distinct,centrally located nucleoli.Sometimes Reed-Sternberg-like giant cells were encountered.Immunohistochemically,the tumor calls were positive for CD21and CD35,and were also positive for EBV-encoded RNA by in situ hybirdization.Conclusions The hepatic IPT-like FDCT is a rare neoplasm.The penal of immunohistochemical markers and EBER are necessary for the final diagnosis of these tumors because of the complexity and similarity of different tumors of this kind.