特发性腹膜后纤维化2例临床病理观察

Idiopathic retroperitoneal fibrosis:a clinicopathologic analysis of 2 cases

目的分析特发性腹膜后纤维化(IRF)的临床病理特点及病因。方法回顾性分析2例IRF的临床资料、组织形态及免疫组化染色。结果 2例IRF的年龄分别为31岁和69岁,临床均有一侧腰背痛和不适,其中1例伴有恶心和呕吐。CT示腹膜后肿块,包绕周围大血管、神经或输尿管。病理改变主要为纤维组织增生及慢性炎细胞浸润。组织形态和免疫组化排除了其他疾病继发和肿瘤的可能性。结论 IRF是特发性腹膜后纤维组织增生性病变,少见。CT示腹膜后包绕周围大血管和输尿管的肿块,病理改变无特异性,需要组织形态上首先排除其他疾病继发累及和肿瘤。

Objective To analyze the etiopathogenesis and the clinicopathologic features of idiopathic retroperitoneal fibrosis（IRF）.Methods Clinical data and HE staining were analyzed in 2 cases of IRF,and immunohistochemistry（IHC） were carried out in 1 cases.Follow-up date was available in 1 case of them.Results The age was 31 years and 69 years,respectively.All of them had a troublesome backache.One of them had concomitantly vomiting.Computed tomography（CT） revealed a plaque in retroperitoneal region.The pathological change was fibrosis and the infiltration of chronic inflammatory cells.After HE and immunohistochemital staining were analyzed,the possibility of tumors and the secondary involvement of other diseases were excluded.More viewpoints considered it was in association with autoimmune and abnormal immunoglobulin IgG4.A part of the cases revealed a prominent rise of IgG4 in periphery blood and numerous IgG4-positive plasma cell infiltrates.Conclusions IRF is rare,and it is a disease which arises from retroperitoneal fibrosis without definite pathogenesis.CT reveals a plaque encompassing the large vessels,nerves or ureters.The pathologic change is nonspecific.It is important to exclude secondary involvement of other disease and tumours before diagnosing IRF.