本文選題：隆突性皮膚纖維肉瘤　切入點：臨床分析　出處：《大連醫(yī)科大學(xué)》2017年碩士論文　論文類型：學(xué)位論文

【摘要】：目的:隆突性皮膚纖維肉瘤(Dermatofibrosarcoma protuberans,DFSP)是一種低度惡性的軟組織纖維肉瘤,其生長緩慢,具有一定局限性及侵襲性。該病病因不明,常起源于皮內(nèi)纖維組織細(xì)胞,可侵及皮下組織,極易原位復(fù)發(fā)、卻很少轉(zhuǎn)移。腫瘤多表現(xiàn)為無痛性結(jié)節(jié)或腫塊,局部可見瘢痕樣或萎縮性斑塊,局部表面可見破潰、結(jié)痂,質(zhì)地堅硬。瘤體形狀多不規(guī)則,個別呈"分葉狀",有或無外包膜,切面多為灰白色呈魚肉狀。典型DFSP病理組織學(xué)表現(xiàn)為梭形腫瘤細(xì)胞呈席紋狀或車輻狀排列,免疫組化染色表現(xiàn)為CD34、Vimentin染色陽性,CK、S-100蛋白染色陰性。臨床以廣泛擴(kuò)大切除術(shù)或Mohs顯微外科手術(shù)(Mohs micrographic surgery,MMS)為主,輔助治療包括放射療法(Radiation Therapy,RT),傳統(tǒng)化療和分子靶向治療等。本文對30例DFSP患者的臨床資料整合統(tǒng)計,進(jìn)行相關(guān)臨床分析,目的在于通過臨床表現(xiàn)結(jié)合病理組織學(xué)檢查及免疫組化染色檢查,早期確診DFSP,并與臨床其他皮膚纖維瘤及類似皮膚腫瘤相鑒別,以進(jìn)一步降低臨床誤診率,同時強(qiáng)調(diào)規(guī)范手術(shù)治療的重要性,探索降低術(shù)后復(fù)發(fā)率的新輔助治療手段。方法:篩選1996年至2015年來收治我院的大連地區(qū)隆突性皮膚纖維肉瘤(DFSP)患者的臨床資料,回顧性分析具有完整病理組織學(xué)檢查、免疫組化染色及診治經(jīng)過的30例患者的病歷及病理資料。統(tǒng)計分析患者首次發(fā)病年齡、性別、病程長短、發(fā)病部位、腫瘤個數(shù)(單發(fā)或多發(fā))、腫瘤最大直徑等。其中男性15例,女性15例,男女比例1:1。首次發(fā)病年齡最小5歲,最大79歲,平均年齡41歲。用一般統(tǒng)計學(xué)分析方法,對入組病例病理組織學(xué)、免疫組化染色檢查結(jié)果及治療方法(手術(shù)為主)進(jìn)行分析。結(jié)果:本文30例DFSP患者術(shù)前早期診斷為其他病變?yōu)?0%以上。治療過程中均具有相應(yīng)病理組織學(xué)檢查結(jié)果,具體表現(xiàn)為典型梭形腫瘤細(xì)胞呈席紋狀、車輻狀排列。免疫組化染色結(jié)果具體表現(xiàn)為CD34、vimentin陽性率為100%,SMA均陰性,CK、S-100蛋白均陰性。本文30例患者均施行手術(shù)切除治療,均未施行術(shù)前、術(shù)中及術(shù)后相關(guān)輔助治療干預(yù),其中16例施行局部切除手術(shù)治療(切緣距瘤體1cm～2cm),占53.3%,其中復(fù)發(fā)10例(62.5%);其余14例施行廣泛擴(kuò)大切除手術(shù)治療(切緣距瘤體≥3cm),占46.7%,其中復(fù)發(fā)2例(14.3%)。結(jié)論:早期DFSP臨床容易與其他皮膚纖維瘤及類似皮膚腫瘤等病變相混淆,通過臨床表現(xiàn)結(jié)合組織病理學(xué)檢查及免疫組化染色檢查,可早期明確診斷。規(guī)范化的廣泛擴(kuò)大切除術(shù)或以Mohs技術(shù)為基礎(chǔ)的前提下,輔以合理的綜合治療,如放射療法(RT),傳統(tǒng)化療和分子靶向治療等,會在一定程度上降低術(shù)后復(fù)發(fā)率、改善患者預(yù)后。
[Abstract]:Objective: Dermatofibrosarcoma protuberanssarcoma (DFSPP) is a low-grade malignant soft tissue fibrosarcoma with slow growth and limited invasion. It is easy to recur in situ, but rarely metastasizes. The tumor is characterized by painless nodules or masses, scarring or atrophic plaques on the local surface, crumbling, scabbing, hard texture, and irregular shape of the tumor. Some of them were "lobulated", with or without outer capsule, and the cut surface was mostly grayish white and fish-like. Typical DFSP histopathological findings showed that spindle tumor cells were arranged in the shape of matte striate or carousel. Immunohistochemical staining showed that CD34-Vimentin positive staining was negative for CKS-100 protein. Extensive excision or Mohs microsurgery were the main clinical manifestations. Adjuvant therapy includes radiation therapy, traditional chemotherapy and molecular targeted therapy. The clinical data of 30 patients with DFSP were analyzed. The aim of this study was to make early diagnosis of DFSPs by clinical manifestations, histopathological examination and immunohistochemical staining, and to differentiate DFSPs from other skin fibromas and similar skin tumors, so as to further reduce the rate of misdiagnosis. At the same time, the importance of standardized surgical treatment was emphasized, and the new adjuvant treatment method was explored to reduce the postoperative recurrence rate. Methods: the clinical data of patients with DFSP in Dalian area from 1996 to 2015 were selected. Retrospective analysis was performed on 30 patients with complete histopathological examination, immunohistochemical staining and diagnosis and treatment. The age, sex, duration and location of the first onset of the disease were statistically analyzed. The number of tumors (single or multiple, maximum diameter of tumor, etc.) 15 male and 15 female, the ratio of male to female was 1: 1.The minimum age of first onset was 5 years old, the maximum age was 79 years, the average age was 41 years. Histopathology of incoming cases, Results: 30 cases of DFSP were diagnosed as other lesions more than 80% in the early stage before operation. The results of immunohistochemical staining showed that the positive rate of CD34 vimentin was 100 and SMA negative, and CKS-100 protein was negative. The related adjuvant therapy intervention during and after operation, Local excision was performed in 16 cases (1 cm ~ 2 cm ~ (-1) cm from the margin of resection, accounting for 53.3%), and recurrence in 10 cases (62.5%). The other 14 cases were treated with extensive excision (margin 鈮，

本文編號：1559320