摘要
目的探讨胸膜外孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理学特征、鉴别诊断、治疗及预后.方法收集5例EPSFT的临床病理资料,并复习相关文献.结果男1例,女4例,平均发病年龄(45.8±9.2)岁.3例为经典型SFT,1例脂肪瘤样型SFT,1例MSFT.肿瘤界清有包膜,灰白色,质地软韧不一.镜检瘤细胞呈短梭形或卵圆形,胞界不清,染色质匀细,核分裂象多少不等.瘤组织呈疏密不一,呈血管外皮瘤样及无结构无模式性生长等多种生长方式.间质胶原纤维多少不等.脂肪瘤样型SFT中可见成熟脂肪组织,与梭形瘤细胞随意掺杂.恶性SFT由经典型SFT及肉瘤性成分混合,后者呈低级别纤维肉瘤、多形性未分化肉瘤改变.5例SFT均显示瘤细胞STAT6弥漫阳性,Vim、CD99、Bcl-2不同程度阳性,CD34阳性者4例,阴性1例.经典型和脂肪瘤样型SFT中,Ki67增殖指数为10%以下,MSFT在50%以上.结论 EPSFT是一种纤维母细胞/肌纤维母细胞起源的间叶性肿瘤,生物学行为难以预料.该肿瘤组织形态多样,结合免疫组化可确诊.要与多种梭形细胞软组织肿瘤和瘤样病变鉴别.
Objective To discuss the clinicopathological features, differentiatial diagnosis, therapy and prognosis of extrapleural solitary fibrous tumor (SFT). Methods The clinicopathological data of 5 patients with SFT were collected and relevant literatures were reviewed. Results Among these cases, one was male and 4 cases were female. The average was (45.8±9.2) years old. There were 3 cases of typical SFT, 1 case of lipomatoid SFT and 1 case of malignant SFT. They had distinct boundaries or with a capsule, whitish, with soft or rubbery-like texture. Microscopic examination showed the tumor cells were short fusiform or ovoid, with unclear cell boundaries, uniform chromatin, and different mitotic figures. These tumours alternated with hypercellular and hypocellular area. With vascular epithelioma-like and patternless-pattern and so on. Interstitial collagen fibers varied in different cases. Mature adipose tissue could be seen in lipomatoid SFT, which was randomly mixed with spindle cells. Malignant SFT mixed of classic SFT and sarcoma component. The latter showed pleomorphic undifferentiated sarcoma and low grade fibrosarcoma. These SFTs cells demonstrated STAT6 diffuse positive . Vim, CD99 and Bcl-2 showed positive in varying degrees. CD34 was positive in 4 cases, and negative in 1 case. The Ki-67 proliferation index was less than 10% in typical SFT and lipomatoid SFT. While it' s more than 50% in MSFT. Conclusion EPSFT is a kind of mesenchymal tumor originated from fibroblasts and myofibroblasts, which biological behavior is unpredictable. The tumor has multiple histological features and can be diagnosed by immunohistochemistry. It should be distinguished from a variety of spindle cell soft tissue tumors and tumor-like lesions.
作者
王志敢
张娜
谢晋予
刘畅
Wang Zhigan;Zhang Na;Xie Jinyu;Liu Chang(Department of Pathology, Changsha Central Hospital, Changsha, Hunan, 410004, China)
出处
《当代医学》
2019年第25期50-54,共5页
Contemporary Medicine
关键词
孤立性纤维性肿瘤
胸膜外
复发
恶性
免疫组化
鉴别诊断
治疗
预后
Solitary fibrous tumor
Extrapleural
Recur
Malignent
Immunohlistochemistry
Differentiatial diagnosis
Therapy
Prognosis
