钙化性纤维性肿瘤4例临床病理学分析

Calcifying fibrous tumor:a clinical and pathological analysis of 4 cases

目的探讨钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)的病理形态学特点、免疫组化表达及鉴别诊断。方法对4例CFT进行免疫组化检测并文献复习。结果本组患者年龄19～33岁,发病部位分别为胃、纵膈、大网膜及肠系膜根部。镜下可见在大量胶原化的纤维组织内伴有钙化或砂砾体形成,淋巴细胞、浆细胞散在浸润。免疫组化标记瘤细胞表达vimentin,而CD117、CD34、CD99、desmin、S-100蛋白、SMA、actin、NFP、ALK1、ER、PR、CK、BCL-2及Ki-67均阴性。结论 CFT是一种极少见的良性纤维性肿瘤,其诊断需要结合年龄、组织学形态及免疫组化等特点,还需与钙化性腱膜纤维瘤、钙化性肉芽肿、纤维瘤病、胃肠道间质瘤、炎性肌纤维母细胞瘤、结节性筋膜炎和淀粉样瘤等相鉴别。

Purpose To study the pathological characteristics,immunophenotype and differential diagnosis of calcifying fibrous tumor（CFT）.Methods Four cases of calcifying fibrous tumor were observed by histopathological and immunohistochemical methods.Results The age of patients with CFT in this group ranged from 19 to 33 years.The four tumor located in stomach,mediastinum,greater omentum and radix of mesentery,respectively.Histopathologically,CFT consisted of abundant collagenous fiber,accompanied by the calcification or psammomatous body and scattered lymphocyte and plasma cell infiltration.Immunohistochemically,all the case expressed vimentin,but negative for CD117,CD34,CD99,desmin,S-100 protein,SMA,actin,NFP,ALK1,ER,PR,CK,BCL-2 and Ki-67.Conclusion Calcifying fibrous tumor is a very rare benign fibrous tumor.The diagnosis of CFT should be based on age,histopathological changes and immunohistochemical findings.CFT should be distinguished from other tumors or tumor-like lesions,such as calcifying aponeurotic fibroma,calcifying granuloma,fibromatosis,gastrointestinal stromal tumor,inflammatory myofibroblastic tumor,nodular fasciitis and amyloid tumor.