30例隆突性皮肤纤维肉瘤的临床分析

发布时间：2018-03-03 03:18

  本文选题：隆突性皮肤纤维肉瘤　切入点：临床分析　出处：《大连医科大学》2017年硕士论文　论文类型：学位论文

【摘要】：目的:隆突性皮肤纤维肉瘤(Dermatofibrosarcoma protuberans,DFSP)是一种低度恶性的软组织纤维肉瘤,其生长缓慢,具有一定局限性及侵袭性。该病病因不明,常起源于皮内纤维组织细胞,可侵及皮下组织,极易原位复发、却很少转移。肿瘤多表现为无痛性结节或肿块,局部可见瘢痕样或萎缩性斑块,局部表面可见破溃、结痂,质地坚硬。瘤体形状多不规则,个别呈"分叶状",有或无外包膜,切面多为灰白色呈鱼肉状。典型DFSP病理组织学表现为梭形肿瘤细胞呈席纹状或车辐状排列,免疫组化染色表现为CD34、Vimentin染色阳性,CK、S-100蛋白染色阴性。临床以广泛扩大切除术或Mohs显微外科手术(Mohs micrographic surgery,MMS)为主,辅助治疗包括放射疗法(Radiation Therapy,RT),传统化疗和分子靶向治疗等。本文对30例DFSP患者的临床资料整合统计,进行相关临床分析,目的在于通过临床表现结合病理组织学检查及免疫组化染色检查,早期确诊DFSP,并与临床其他皮肤纤维瘤及类似皮肤肿瘤相鉴别,以进一步降低临床误诊率,同时强调规范手术治疗的重要性,探索降低术后复发率的新辅助治疗手段。方法:筛选1996年至2015年来收治我院的大连地区隆突性皮肤纤维肉瘤(DFSP)患者的临床资料,回顾性分析具有完整病理组织学检查、免疫组化染色及诊治经过的30例患者的病历及病理资料。统计分析患者首次发病年龄、性别、病程长短、发病部位、肿瘤个数(单发或多发)、肿瘤最大直径等。其中男性15例,女性15例,男女比例1:1。首次发病年龄最小5岁,最大79岁,平均年龄41岁。用一般统计学分析方法,对入组病例病理组织学、免疫组化染色检查结果及治疗方法(手术为主)进行分析。结果:本文30例DFSP患者术前早期诊断为其他病变为80%以上。治疗过程中均具有相应病理组织学检查结果,具体表现为典型梭形肿瘤细胞呈席纹状、车辐状排列。免疫组化染色结果具体表现为CD34、vimentin阳性率为100%,SMA均阴性,CK、S-100蛋白均阴性。本文30例患者均施行手术切除治疗,均未施行术前、术中及术后相关辅助治疗干预,其中16例施行局部切除手术治疗(切缘距瘤体1cm～2cm),占53.3%,其中复发10例(62.5%);其余14例施行广泛扩大切除手术治疗(切缘距瘤体≥3cm),占46.7%,其中复发2例(14.3%)。结论:早期DFSP临床容易与其他皮肤纤维瘤及类似皮肤肿瘤等病变相混淆,通过临床表现结合组织病理学检查及免疫组化染色检查,可早期明确诊断。规范化的广泛扩大切除术或以Mohs技术为基础的前提下,辅以合理的综合治疗,如放射疗法(RT),传统化疗和分子靶向治疗等,会在一定程度上降低术后复发率、改善患者预后。
[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，

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