软组织血管纤维瘤的临床病理学特征分析

Clinicopathologic analysis of soft tissue angiofibroma

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 is a kind of soft tissues tumor. The objective of this study was to investigate the clinicopathologic characteristic,differential diagnosis and biological behavior of soft tissue angiofibroma. METHODS Seven cases of soft tissue angiofibroma diagnosed were reviewed. The clinical, pathologic and immunohisto- chemical profiles were evaluated. RESULTS There were 3 males and 4 females at diagnosis with age ranging from 20 years and 61 years（median,43 years）. The tumors presented most commonly as a slowly growing painless mass located in the soft tissue of upper extremity, head and neck, and lower extremity often adjacent to or arising from joint-related structures. The lesions were treated by complete surgical resection. All patients were followed up for 4 months to 8 years and no one showed recurrence. On gross examination,the tumor varied from 2 cm to 12 cm in greatest dimension. They were well-circumscribed,3 of them being apparently encapsulated. Consistency was firm, elastic or myxoid. A glistening cut surface can been investigated. Microscopically, the tumor was composed of bland-looking spindle cells with numerous, branching blood vessels, embedded in a myxiod or collagenous, stromal background with some scattered mast cells and chronic inflammatory cells. Also present were dilated, sometimes branching, hemangio-pericytoma-like blood vessels. The neoplastic cells showed focal degenerative changes characterized. Mitotic counts were 〈1/10 HPF in 7 cases. Immunohis- to-chemically, all cases diffuse and strong staining for vimentin. SMA and Desmin were detected focally in tumor cells. The vascular pattern was highlighted by CD34,CD31 and Fli-1 staining of endothelial cells,meanwhile, Bcl-2, β-catenin, Calponin, CD99, AE1/AE3, MSA myogenin, MyoD1 were negative in all cases, with Ki-67 proliferation index % 1 ~. CONCLUSIONS Soft tissue angiofibroma is a recently described neoplasm that typically presents as a slowly growing, painless mass in the soft tissues of extremities,having the characteristics of a well circumscribed fibro/fibroblastic tumor with unique vascular features. Treatment is complete excision. The pathologic differential diagnosis for this leision include both benign and magiinant leisions,especially to prevent misdiagnosis of a low-grade sarcoma. Increased awareness of the clinopathological features and immunophenotypes of soft tissue angiofibroma in helpful in avoiding misdiagnosing the disease.