罕见的肝脏炎性肌纤维母细胞瘤的临床病理分析

目的研究肝脏炎性肌纤维母细胞瘤(hepatic inflammatory myofibroblastic tumor,hepatic IMT)的临床特点,以提高对该病的认识和诊疗水平。方法收集5例患者的临床资料,分析和总结其主要的临床病理特点。结果肝脏单发者4例,多部位受累者1例。临床症状包括发热、乏力、纳差、肿块局部疼痛等。可伴有贫血、纤维蛋白原和肝酶增高。病理检查示梭形肿瘤细胞排列成束状或编织状分布于胶原性基质中,伴淋巴细胞、浆细胞浸润,肿瘤细胞表达波形蛋白Vim(5/5)、平滑肌肌动蛋白SMA(5/5)和肌特异性蛋白MSA(5/5)。全部患者行肿瘤完整切除。随访11~65个月,无复发、转移、死亡。结论肝脏IMT的临床表现无特异性,病理学检查和免疫组化对诊断意义较大,手术完整切除是其有效的治疗方法。

Analysis of clinical and pathological characteristics of rare hepatic inflammatory myofibroblastic tumor

Objective To investigate the clinical features of hepatic inflammatory myofibroblastic tumor （IMT）.Methods The clinical data of 5 cases of hepatic IMT,and the clinicopathologic features were analyzed.Results In 4 cases,tumor only grew in the liver.In 1 case,tumor grew in multiple locations.Symptoms included fever,fatigue,anorexia,pain in the focal area,etc.Anemia,high fibrinogen and liver enzymes could be detected.Histologically,spindle-shaped tumor cells displayed in a bunch manner in collagenous stroma with inflammatory infiltration composed of plasma cells and lymphocytes.Immunohistochemically,tumor cells showed strong positive staining for Vim （5/5）,SMA （5/5） and MSA （5/5）.Tumors were all completely resected.After 11 ～ 65 months follow up,all patients were alive without recurrence after the surgery.Conclusion Symptoms of hepatic IMT are not specific.Pathological and histological examinations contribute greatly to the diagnosis.Complete surgical resection is an effective therapeutic method for the hepatic IMT patients.