15例腹腔炎性肌纤维母细胞瘤的临床病理分析

目的报道和总结腹腔炎性肌纤维母细胞瘤的临床特点,以提高医生对该病的诊疗水平。方法收集15例患者的临床资料,分析和总结其主要的临床病理特点。结果本组病例包括男性8例,女性7例,发病年龄为4～63岁。发生部位包括肝脏(5例)、膀胱(3例)、肠系膜(2例)、胃(1例)、脾(1例)、直肠(1例)、肾上腺(1例)、腹膜腔(1例)。多部位受累者2例。临床症状包括发热、乏力、纳差、肿块局部疼痛等。常伴有贫血,纤维蛋白原增高。病理学检查可见胶原性基质中呈束状排列的梭形细胞伴淋巴细胞、浆细胞等炎性细胞浸润。免疫组化显示梭形细胞表达波形蛋白Vim(15/15)、平滑肌肌动蛋白SMA(15/15)和肌特异性蛋白MSA(15/15),部分表达ALK(6/15)、Calponin(6/15)、Desmin(5/15)、CK(4/15)。13例行肿瘤完整切除,1例行肿瘤部分切除,1例仅行活检。随访11～65个月,13例术后无瘤生存。结论腹腔炎性肌纤维母细胞瘤是一种罕见的间叶源性肿瘤。累及部位不同导致临床表现多种多样。诊断主要依靠病理学检查和免疫组化检测。手术完整切除是大多数患者的有效治疗方法。

Clinicopathologic Characteristics of Abdominal Inflammatory Myofibroblastic Tumor in 15 Cases

Objective To summarize the clinical features of abdominal inflammatory myofibroblastic tumor(IMT).MethodsThe clinical data of 15 cases of abdominal IMT,and the clinicopathologic features were analyzed.Results The patients included 8 men and 7 women,with age ranging from 4 to 63 years old.The tumor located in the liver(5 cases),gallbladder(3 cases),mesentery(2 cases),stomach(1 case),spleen(1 case),rectum(1 case),adrenal gland(1 case),and abdominal cavity(1 case).In 2 cases,tumor grew in multiple locations.Symptoms included fever,fatigue,anorexia,pain in the focal area,etc.Anemia and high fibrinogenemia were common.Histologically,spindle-shaped cells proliferated in a bunch manner in collagenous stroma with inflammatory infiltration primarily composed of plasma cells and lymphocytes.Immunohistochemically,spindle-shaped cells showed strong positive staining for Vim(15/15),SMA(15/15)and MSA(15/15),partial positive staining for ALK(6/15),Calponin(6/15),Desmin(5/15)and CK(4/15).In 13 cases,tumors were completely resected.In one case,tumor was partly resected.One case just underwent biopsy.After follow-up for 11-65 months,13 patients were alive without recurrence after the surgery.Conclusion Abdominal IMT,a rare kind of tumor,origins from mesenchymal tissue.Symptoms vary according to the different locations of tumor.Diagnosis mainly depends on the pathological and histological examinations.Complete surgical resection is an effective therapeutic method for most IMT patients.