發(fā)布時間：2018-05-18 05:42

  本文選題：血管纖維瘤 + 良性��； 參考：《中華腫瘤防治雜志》2017年02期

【摘要】：目的軟組織血管纖維瘤(soft tissue angiofibroma,STAF)為新近報道的軟組織腫瘤,對其臨床及組織學(xué)形態(tài)尚未完全明了。為此,有必要收集更多的病例,以深入探討其臨床病理學(xué)特征、鑒別診斷及生物學(xué)行為。方法收集2008-01-05-2016-07-14湖南省人民醫(yī)院(2例)、佛山中醫(yī)院(3例)、深圳市龍華新區(qū)中心醫(yī)院(1例)和撫順市新?lián)釁^(qū)中醫(yī)院(1例)共7例STAF,對其臨床特征、病理形態(tài)和免疫學(xué)表型進行分析。結(jié)果7例病例中男3例,女4例,年齡20~61歲,中位年齡為43歲。臨床多表現(xiàn)為無痛性腫塊,分別位于后頸部頭皮下、左膝關(guān)節(jié)、右外踝、右肘部、前額皮下、左足背及右大腿。腫塊部分與周圍組織及關(guān)節(jié)囊有粘連。腫塊均完整切除,術(shù)后隨訪4個月至8年,無1例復(fù)發(fā)。腫瘤直徑2~12cm,境界清楚,切面灰白、灰黃,質(zhì)地韌或者硬,部分切面有光澤帶黏液感。瘤組織主要由大量血管及短梭至卵圓形核的梭形細胞組成,分布于比例不等的黏液樣或者膠原化的基質(zhì)中,梭形細胞形態(tài)溫和、大小相對一致,局部可見核的不典型性和核的退變,核分裂1/10HPF。瘤內(nèi)血管主要由大量薄壁分支狀血管構(gòu)成,也可見較大的厚壁血管及呈鹿角樣形態(tài)的血管外皮瘤樣結(jié)構(gòu),伴有肥大細胞及慢性炎癥細胞浸潤。組織學(xué)上需要與腱鞘纖維瘤、黏液性纖維肉瘤和低度惡性纖維黏液樣肉瘤等鑒別。免疫組化檢測結(jié)果顯示,7例患者腫瘤均彌漫表達Vimentin,血管內(nèi)皮細胞表達CD31、CD34及FLI-1,顯示腫瘤中血管的結(jié)構(gòu)和分布特征,SMA在3例患者中局限性表達,DES在4例患者中局限性表達,Ki-671%,AE1/AE3、MSA、Bcl-2、β-catenin、Calponin、CD99、Myogenin和MyoD1均為陰性,Ki-67陽性指數(shù)為1%。結(jié)論STAF是一種好發(fā)于肢端的無痛性緩慢增長的具有獨特形態(tài)學(xué)特征的良性軟組織腫瘤,主要由程度不等的梭型纖維母細胞及顯著分支狀血管構(gòu)成,形態(tài)學(xué)上需與富含梭型纖維母細胞及血管的多種病變?nèi)珉烨世w維瘤、黏液性纖維肉瘤、低度惡性纖維黏液樣肉瘤等相鑒別。臨床上手術(shù)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.
【作者單位】： 湖南省人民醫(yī)院病理科;廣州中醫(yī)藥大學(xué)附屬佛山市中醫(yī)院病理科;深圳市龍華新區(qū)人民醫(yī)院病理科;撫順市中醫(yī)院病理科;
【基金】：佛山市十三五重點專科建設(shè)項目(FSZDZK135018) 佛山市中醫(yī)院綜合防治腫瘤創(chuàng)新科研平臺建設(shè)項目,佛山市科技創(chuàng)新專項資金(2014AG1008)
【分類號】：R738.6

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