软组织血管纤维瘤的临床病理学特征分析

发布时间：2018-05-18 05:42

本文选题：血管纤维瘤 + 良性　；参考：《中华肿瘤防治杂志》2017年02期

【摘要】：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-671%,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。
[Abstract]:Objective soft tissue angiofibroma (tissue) is a newly reported soft tissue tumor. Therefore, it is necessary to collect more cases in order to explore the clinicopathological features, differential diagnosis and biological behavior. Methods A total of 7 cases of STAF were collected from Hunan Provincial people's Hospital, Foshan traditional Chinese Medicine Hospital (3 cases), Central Hospital of Longhua District (Shenzhen City, 1 case) and Fushun Xinfu District Hospital (1 case). The clinical features, pathological morphology and immunophenotype were analyzed. Results there were 3 males and 4 females, aged 20 to 61 years, with a median age of 43 years. The clinical manifestations were painless masses, located under the scalp of the posterior neck, left knee joint, right lateral malleolus, right elbow, subcutaneous forehead, left back of foot and right thigh. The mass had adhesion with surrounding tissue and articular capsule. The masses were resected completely and followed up for 4 months to 8 years. No recurrence occurred. The tumor is 22 cm in diameter, with clear boundary, grayish white, grayish yellow, tough or hard texture, and part of the cut surface with gloss and mucus. The tumor tissue is mainly composed of a large number of blood vessels and spindle cells from the short spindle to the oval nucleus, distributed in different proportions of mucoid or collagenized matrix. The spindle cells are mild in morphology and relatively uniform in size. The heterogeneity of the locally visible nuclei and the degeneration of the nuclei, and the mitosis of 1 / 10 HPF. The intratumoral blood vessels were mainly composed of a large number of parenchyma branched vessels. The thick-walled vessels and the angiopericytoma-like structure of antlers were also found, accompanied by the infiltration of mast cells and chronic inflammatory cells. Histologically, it needs to be distinguished from tendon sheath fibroma, myxofibrosarcoma and low grade malignant fibrous myxoid sarcoma. The results of immunohistochemical staining showed that Vimentin was expressed diffusely in 7 patients, CD31pCD34 and FLI-1were expressed in vascular endothelial cells, and the structure and distribution of blood vessels in the tumor were observed. The localization of SMA expression of des was found in 4 patients. The expression of Ki-671and AE1 / AE3MSA-2, 尾 -cateninine Calponine CD99 Myogenin and MyoD1 were negative and Ki-67 positive index was 1. Conclusion STAF is a kind of benign soft tissue tumor with unique morphological features, which is painless and slowly growing at the extremities. It is mainly composed of fusiform fibroblasts with different degrees and significant branching vessels. Morphologically, it should be distinguished from many diseases, such as tendon sheath fibroma, myxofibrosarcoma and low-grade malignant fibroid sarcoma, which are rich in fusiform fibroblasts and blood vessels. Clinical complete resection can be cured.
【作者单位】：湖南省人民医院病理科;广州中医药大学附属佛山市中医院病理科;深圳市龙华新区人民医院病理科;抚顺市中医院病理科;
【基金】：佛山市十三五重点专科建设项目(FSZDZK135018) 佛山市中医院综合防治肿瘤创新科研平台建设项目,佛山市科技创新专项资金(2014AG1008)
【分类号】：R738.6

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