论文部分内容阅读
目的软组织血管纤维瘤(soft tissue angiofibroma,STAF)为新近报道的软组织肿瘤,对其临床及组织学形态尚未完全明了。为此,有必要收集更多的病例,以深入探讨其临床病理学特征、鉴别诊断及生物学行为。方法收集2008-01-05-2016-07-14湖南省人民医院(2例)、佛山中医院(3例)、深圳市龙华新区中心医院(1例)和抚顺市新抚区中医院(1例)共7例STAF,对其临床特征、病理形态和免疫学表型进行分析。结果7例病例中男3例,女4例,年龄20~61岁,中位年龄为43岁。临床多表现为无痛性肿块,分别位于后颈部头皮下、左膝关节、右外踝、右肘部、前额皮下、左足背及右大腿。肿块部分与周围组织及关节囊有粘连。肿块均完整切除,术后随访4个月至8年,无1例复发。肿瘤直径2~12cm,境界清楚,切面灰白、灰黄,质地韧或者硬,部分切面有光泽带黏液感。瘤组织主要由大量血管及短梭至卵圆形核的梭形细胞组成,分布于比例不等的黏液样或者胶原化的基质中,梭形细胞形态温和、大小相对一致,局部可见核的不典型性和核的退变,核分裂<1/10HPF。瘤内血管主要由大量薄壁分支状血管构成,也可见较大的厚壁血管及呈鹿角样形态的血管外皮瘤样结构,伴有肥大细胞及慢性炎症细胞浸润。组织学上需要与腱鞘纤维瘤、黏液性纤维肉瘤和低度恶性纤维黏液样肉瘤等鉴别。免疫组化检测结果显示,7例患者肿瘤均弥漫表达Vimentin,血管内皮细胞表达CD31、CD34及FLI-1,显示肿瘤中血管的结构和分布特征,SMA在3例患者中局限性表达,DES在4例患者中局限性表达,Ki-67<1%,AE1/AE3、MSA、Bcl-2、β-catenin、Calponin、CD99、Myogenin和MyoD1均为阴性,Ki-67阳性指数为<1%。结论STAF是一种好发于肢端的无痛性缓慢增长的具有独特形态学特征的良性软组织肿瘤,主要由程度不等的梭型纤维母细胞及显著分支状血管构成,形态学上需与富含梭型纤维母细胞及血管的多种病变如腱鞘纤维瘤、黏液性纤维肉瘤、低度恶性纤维黏液样肉瘤等相鉴别。临床上手术完整切除可治愈。
Objective Soft tissue angiofibroma (STAF) is a newly reported soft tissue tumor, and its clinical and histological features have not yet been fully understood. Therefore, it is necessary to collect more cases to further explore its clinical and pathological features, differential diagnosis and biological behavior. Methods Hunan Provincial People’s Hospital (2 cases), Foshan Hospital of Traditional Chinese Medicine (3 cases), Longhua New Area Central Hospital (1 case) and Fushun Xin Fu District Chinese Medicine Hospital (1 case) Cases) A total of 7 cases of STAF, its clinical features, pathological morphology and immunological phenotypes were analyzed. Results There were 3 males and 4 females in 7 cases, ranging in age from 20 to 61 years with a median age of 43 years. Clinical manifestations of painless mass, respectively, located in the scalp under the neck, left knee, right lateral malleolus, right elbow, the frontal subcutaneous, left dorsal and right thigh. Mass part of the surrounding tissue and joint capsule adhesions. Tumors were completely removed, followed up for 4 months to 8 years, no recurrence in 1 case. Tumor diameter of 2 ~ 12cm, the realm of clear, cut gray, yellowish, tough texture or hard, part of the glossy glossy with mucus. Tumor mainly composed of a large number of blood vessels and short shuttle to the oval nucleus of spindle cells, distributed in varying proportions of mucoid or collagen matrix, spindle cells in a modest, relatively uniform size, the local visible nuclear Typical and nuclear degeneration, mitotic <1 / 10HPF. The intratumoral vessels are mainly composed of a large number of thin-walled branched vessels, and also large, thick-walled vessels and antler-like hemangioperitoid-like structures with infiltration of mast cells and chronic inflammatory cells. Histological needs and tendon sheath fibroma, mucinous fibrosarcoma and low grade malignant fibrous myxoid sarcoma and other identification. The results of immunohistochemistry showed that in all 7 cases, Vimentin was diffusely expressed in tumor and CD31, CD34 and FLI-1 were expressed in vascular endothelial cells. The structure and distribution of blood vessels in the tumor were shown. SMA was localized in 3 cases and DES The expression of Ki-67 was less than 1% in all 4 cases. The positive rates of Ki-67 positive index were less than 1% for AE1 / AE3, MSA, Bcl-2, β-catenin, Calponin, CD99, Myogenin and MyoD1. Conclusions STAF is a kind of benign soft tissue tumor with unique morphological characteristics that develops slowly and without pain in the extremities. It is mainly composed of spindle fibroblasts and prominent branch vessels with varying degrees, which are morphologically related to rich Fusiform fibroblasts and blood vessels with a variety of lesions such as tendon sheath fibroma, mucinous fibrosarcoma, low grade mucinous sarcoma and other fibrous differentiations. Clinically complete surgical resection can be cured.