胸膜外孤立性纤维性肿瘤44例临床病理特征分析

目的:探讨胸膜外孤立性纤维性肿瘤(extrapleural solitary fibrous tumor,ESFT)的临床病理特征、鉴别诊断及预后。方法:对44例ESFT病例行病理学观察及免疫组化染色,结合临床及随访资料进行分析。结果:良性35例,恶性9例。ESFT组织学形态多样,典型者见梭形细胞呈无模式(patternless)排列,伴有粗大瘢痕样的胶原及血管外皮瘤样结构。免疫组化染色Vimentin、CD34、Bcl-2、CD99、SMA阳性率分别为100%、93%、79%、67%和6%,Desmin、S-100、CD117、Calretinin及EMA均为阴性。随访33例,3例局部复发,无死亡及远处转移。结论:临床病理特征和免疫组化表型有助于ESFT诊断及鉴别诊断。对ESFT患者有必要进行术后长期随访。

Extrapleural solitary fibrous tumors: a clinicopathologic analysis of 44 cases

Objective:To investigate the clinicopathologic characteristics,differential diagnosis and prognosis of extrapleural solitary fibrous tumor(ESFT).Methods: Clinic and follow-up data were analyzed with results of pathologic and immunohistochemical examination of 44 cases of ESFT.Results: In 44 cases of ESFT,35 benign and 9 malignant cases were identified.The histological spectrum of ESFT is broad and characterized by patternless arrangement of bland-looking spindly cells accompanied with thick keloid collagen and haemangiopericytoma-like architecture.The positive rates of Vimentin,CD34,Bcl-2,CD99 and SMA were 100%,93%,79%,67% and 6% respectively.Desmin,S-100,CD117,Calretinin and EMA were all negative.Follow-up information on 33 cases revealed that 3 patients had had local recurrence.There was no death and metastasis.Conclusion: The clinicopathologic features and immunohistochemical profile are helpful to identify ESFT.Long-term clinical follow-up is necessary for all cases of ESFT.