22例隆起性皮肤纤维肉瘤治疗分析

对22例隆起性皮肤纤维肉瘤(DFSP)病例的治疗方案进行回顾性分析,探讨DFSP理想治疗方案。     

隆突性皮肤纤维肉瘤的术后放疗

目的研究放疗在隆突性皮肤纤维肉瘤（DFSP）中的价值。方法回顾性分析1993年1月-2002年12月收治经组织病理学确诊的43例DFSP患者。且手术次数至少1次以上，术后放疗剂量多数为50～60Gy。结果43例患者手术后共复发91次，第一次手术后复发时间多在0．5～7年（39／43），第二次手术后复发时间多在1～24个月（21／39），均行手术＋放疗。中位随访时间37个月，40例（93．0％）达到局部控制，3例（7．0％）局部复发。结论术后放疗能有效降低DFSP的复发率，放疗剂量50-606y较为合适。     

隆突性皮肤纤维肉瘤21例治疗报告

隆突性皮肤纤维肉瘤位于体表,早期易发现,但临床上常因与其他体表良性肿瘤（如皮脂腺囊肿、脂肪瘤）相混淆而被误诊。隆突性皮肤纤维肉瘤切除不彻底极易复发,多次复发可能导致远处转移,但如处理得当,亦可完全治愈。本院1988年～2011年共收治隆突性皮肤纤维肉瘤21例,现报告如下。     

隆突性皮肤纤维肉瘤85例临床分析

目的：探讨隆突性皮肤纤维肉瘤的临床特点和治疗方法。方法：回顾性分析85例隆突性皮肤纤维肉瘤临床诊治过程。结果：85例隆突性皮肤纤维肉瘤行扩大切除术45例，扩大切除加植皮或转移皮瓣修补术34例，术前放疗2例，术后合并放疗10例，全组病例复发率为64．7％，5例死于肿瘤转移，结论：隆突性皮肤纤维肉瘤是皮肤低度恶性肿瘤，复发率高，首次治疗时彻底切除极为关键，外科手术是其主要的治疗方法，治疗作为辅助治疗有一定疗效。     

具有纤维肉瘤变的隆突性皮肤纤维肉瘤

对９例具有纤维肉瘤改变的隆突性皮肤纤维肉瘤进行了分析。该肿瘤以隆突性皮肤纤维肉瘤和纤维肉瘤的组织结构在同一种瘤并存为特点，且肿瘤部位表浅。与经典的ＤＦＳＰ比较：ＤＦＳＰ－ＦＳ的形态指标恶化转移能力增强。     

隆突性皮肤纤维肉瘤42例临床病理分析

目的探讨隆突性皮肤纤维肉瘤(DFSP)的临床病理特点,影响预后的有关因素.方法回顾分析42例DFSP的临床病理特征,并将其分为三个亚型:普通型、粘液型及纤维肉瘤型.结果 DFSP无明显性别差异;20～50岁最多见;好发于躯干;病程为3个月～35年:肿物多为隆起于皮肤的单个结节,瘤体大小为1cm～11cm;瘤组织位于真皮层,典型形态为梭形细胞呈车辐状排列.结论由于DFSP为交界性肿瘤,复发率高,故手术切除距肿瘤边缘要在3cm以上;因纤维肉瘤型DFSP复发率高达73%～89%,故在病理诊断时要注明亚型.     

隆突性皮肤纤维肉瘤的术后治疗

目的 探讨隆突性皮肤纤维肉瘤(DFSP)术后放疗的价值.方法 25例DFSP术后患者接受放疗,DT 2 Gy,每天1次,总剂量DT 50 ～ 70 Gy,共5～7周.结果 25例手术+放疗患者局部控制率92.0％(23/25),局部复发率8.0％(2/25),远处转移率4.0％ (1/25).结论 术后放疗能有效降低DFSP的复发率.     

隆突性皮肤纤维肉瘤18例临床分析

目的　探讨隆突性皮肤纤维肉瘤 (DFSP)术后复发的因素。方法　回顾性分析 18例隆突性皮肤纤维肉瘤诊治过程及复发情况。结果　 7例隆突性皮肤纤维肉瘤行扩大根治切除术 ,复发率为 43 % ;11例行扩大切除加术后局部放疗 ,复发率为 18%。同时DFSP复发与组织学分级显著相关。结论　对隆突性皮肤纤维肉瘤认识不足 ,首次手术切除不彻底 ,扩大根治不规范 ,术后未行放疗及瘤细胞分化程度低是其复发的主要因素。     

隆突性皮肤纤维肉瘤的综合治疗

目的：探讨隆突性皮肤纤维肉瘤的临床特点，治疗方式及影响预后的因素。方法：回顾性分析我院近10年收治的28例隆突性皮肤纤维肉瘤患，均行肿块局部扩大切除，术后两周行^60Go-r线放疗。结果：本组28例均行手术及放疗，随访5－9年，术后复发率为28．6％（8／28）；3年、5年生存率为96．4％（27／28），92．9％（26／28）。结论：隆突性皮肤纤维肉瘤是一种低恶性肿瘤，行肿块局部扩大切除，术后辅助放疗可有效地降低复发率，对提高生存率及生存质量均有积极作用，是隆突性皮肤纤维肉瘤最佳治疗方案。     

乳腺隆突性皮肤纤维肉瘤２例

病例1:患者女性,41岁。因"右乳肿块反复切除3次,再次复发肿块2月伴进行性增大及破溃数天"入院。1993年患者因右乳上方条索状肿块,表面伴红色结节,外院行肿块切除术;2004年右乳切口处长出肿块,约鸡蛋大小,表面仍伴红色结节,于外院行肿块切除术;2006年右乳原切口处再次长出肿块,呈进行性增大,伴皮肤破溃、出血,再次于外院行肿块切除术;上述3次手术后均未行病理检查。专科检查:     

Targeted therapy for dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, cutaneous tumor characterized by aggressive local invasion. Local recurrence after excision is common, especially when negative margins are not achieved. DFSP frequently exhibits translocation of chromosomes 17 and 22, t(17;22). This rearrangement fuses the collagen type Ià1 (COL1A1) gene to the platelet-derived growth factor B-chain (PDGFB) gene. The resultant chimeric gene causes unregulated expression of platelet-derived growth factor leading to abnormal activation of the platelet-derived growth factor receptor (PDGFR) a tyrosine kinase through an autocrine loop. This is believed to be the critical event in DFSP tumorigenesis. Imatinib mesylate is a potent inhibitor of several protein tyrosine kinases, including the PDGFRs. Clinical evidence suggests that imatinib mesylate is a safe and effective treatment in DFSP, especially in cases of recurrent or metastatic disease. Three phase II, multicenter clinical trials are open to further investigate the role of imatinib mesylate in DFSP.     

隆突性皮肤纤维肉瘤的综合治疗

目的　探讨隆突性皮肤纤维肉瘤的临床特点、治疗方式及影响预后的因素。方法　回顾性分析我院近 10年收治的 2 8例隆突性皮肤纤维肉瘤患者 ,均行肿块局部扩大切除 ,术后两周行6 0 Go -r线放疗。结果　本组 2 8例均行手术及放疗 ,随访 5～ 9年 ,术后复发率为 2 8 6% (8 2 8) ;3年、5年生存率分别为96 4% (2 7 2 8)、92 9% (2 6 2 8)。结论　隆突性皮肤纤维肉瘤是一种低恶性肿瘤 ,行肿块局部扩大切除 ,术后辅助放疗可有效地降低复发率 ,对提高生存率及生存质量均有积极作用 ,是隆突性皮肤纤维肉瘤最佳治疗方案     

Chemosurgical reports: myxoid dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a fibroblastic tumor characterized by a high rate of recurrence following conventional surgical treatment. Several different histopathologic patterns exist, with the "cartwheel" pattern the most common. In this report, a patient with the unusual myxoid histopathologic pattern was successfully treated with Mohs surgery.     

Outcomes of surgery for dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass on the trunk and proximal extremity. The tumour grows slowly, typically over years. The standard treatment is wide local excision with at least a 3-cm margin. The local regional recurrence is up to 50%, emphasizing the need for wide margins for local control. A small fraction of DFSP may metastasize, but on histological examination such tumours have features of fibrosarcomas rather than DFSP. HYPOTHESIS: This study was done to review our experience of the time interval to recurrence of DFSP. DESIGN: A retrospective review was undertaken to identify patients with DFSP in our university teaching hospital. METHODS: All patients received their primary surgical treatment in our department between February 1968 and June 2001. Treatment consisted of wide local excision with margins of at least 3 cm. The chi-square test and Fisher's exact test were performed to determine the relationship between recurrence and clinicopathological variables. We evaluated the prognostic variables using the Kaplan-Meier method with log-rank comparison. RESULTS: The median follow-up period was 59 months. The 5 and 10-year disease-free survival (DFS) were 86 and 76%, respectively. The overall recurrence rate was 16.7%. The mean time to recurrence was 38+/-12 months (range 1-100 months). In 30% of those patients with recurrences, the local regional recurrence was after 5 years. CONCLUSION: Wide local excision with good margins decreases local regional recurrences in patients with DFSP. Close surveillance is necessary even beyond 5 years because late recurrences occur.     

甲磺酸伊马替尼治疗晚期隆突性皮肤纤维肉瘤的临床研究

目的 评价晚期隆突性皮肤纤维肉瘤(DFSP)患者应用甲磺酸伊马替尼的疗效和不良反应.方法 2004年11月至2009年10月,应用甲磺酸伊马替尼(400 mg,口服,每日1次)治疗晚期有随访资料的DFSP患者24例,其中二线治疗2例,三线或三线以上治疗22例,对其疗效、不良反应和生存时间进行分析.结果 全组24例患者均可评价疗效,其中完全缓解(CR)8例,占33.3%;部分缓解(PR)10例,占41.7%;稳定(SD)2例,占8.3%;进展(PD)4例,占16.7%.全组患者的疾病控制率为83.3%.获得CR和PR的18例患者的中位缓解时间为5.6个月.疾病控制(CR+PR+SD)者的中位生存时间为30个月,无效(PD)者的中位生存时间为10个月,二者差异有统计学意义(P＜0.001).与甲磺酸伊马替尼治疗相关的不良反应为恶心呕吐(20.8%)、中性粒细胞减少(12.5%)和水肿(8.3%)等,多为轻至中度.结论 甲磺酸伊马替尼治疗中国晚期DFSP患者的有效率高,耐受性好;推荐剂量为400 mg,每日1次.

Abstract：

Objective To evaluate the efficacy, side effects and toxicity of imatinib mesylate in the treatment of patients with locally advanced and/or metastatic dermatofibrosarcoma protuberans (DFSP). Methods Twenty-four cases of advanced DFSP diagnosed by pathology and treated in our hospital from Nov. 2004 to Oct. 2009 were included in this study. The patients were treated with imatinib mesylate (dosage: 400 mg, po, qd) and carefully observed for treatment efficacy, side effects and survival time. There were 2 patients taking the drug as second line therapy, and other 22 patients as third or more than third line therapy. Results The 24 patients were evaluable for the efficacy. There were 8 patients (33.3%) with CR, 10 pts (41.7%) PR, 2 patients (8.3%) SD, and 4 patients (16.7%) PD. The disease control rate (DCR＝CR+PR+SD) was 83.3%. The median response time in 18 cases with CR and PR was 5.6 months. The median survival time in 20 cases with disease control was 30 months, however, that in nonresponse (PD) cases was only 10 months. Side reactions related to imatinib mesylate included nausea and vomiting (20.8%), neutropenia (12.5%), and edema (8.3%). Conclusions Our results are consistent with previous reports in the literature. Imatinib is a safe and effective moleucular target drug used for Chinese. Only mild adverse reactions occur in the treated patients. It is worth using imatinib in the treatment of advanced DFSP patients.     

128例隆突性皮纤维肉瘤临床疗效分析

目的探讨隆突性皮纤维肉瘤的诊断、复发原因及早期扩大切除手术的治疗效果.方法 1969～2001年我院共收治128例隆突性皮肤纤维肉瘤患者,对其临床和病理资料进行回顾性分析.结果 128例隆突性皮肤纤维肉瘤行扩大切除54例,扩大切除加植皮或转移皮瓣修复术56例,局部切除术后行放疗10例.首诊误诊率高达52.2%.全组病例复发率为53.1%,8例死于肿瘤转移.首次手术即时或延期(术后3个月内)行扩大切除者60例,8例(13.3%)复发,明显低于总体复发率53.1%(χ2=28.4,P<0.001).局部切除后行放射治疗10例,2年内4例(40%)复发,与总体复发率相比差异无显著性(χ2=0.642,P>0.05).结论认识不足,首次治疗误诊,局部手术切除范围过于保守是局部复发率高的主要原因;早期三维扩大切除可有效降低隆突性皮肤纤维肉瘤的局部复发率;放射治疗不能降低局部复发率.     

纤维肉瘤型隆突性皮肤纤维肉瘤的临床特征分析

目的:探讨纤维肉瘤型隆突性皮肤纤维肉瘤（fibrosarcomatous dermatofibrosarcoma protuberans,FS-DFSP ）的临床特征、治疗方法及预后因素。方法:收集天津市黄河医院自2004年6 月至2014年6 月收治的经病理学证实的18例FS-DFSP ,分析包括年龄、性别、部位、既往非计划手术次数、肿瘤大小、深浅、末次手术切缘、化疗、复发、转移、生存时间等因素。结果:18例患者均接受手术治疗,17例R 0,1 例R 1,局部复发 2 例,R 1 切缘患者术后放疗。12例患者（肿瘤基底深在、和/或肿瘤最长径≥ 5 cm）术后接受MAID方案化疗,无化疗期间病情进展病例,化疗结束后病情进展（肺转移）3 例,肺转移伴局部复发1 例。2 例接受甲磺酸伊马替尼治疗,疗效为SD。患者的2 年生存率为93% ,5 年生存率为79% 。单因素分析结果显示性别、年龄、部位、肿瘤大小、深度、复发、手术切缘、化疗等因素与总生存时间无关,而既往非计划手术次数和转移与总生存时间相关。多因素分析显示各临床因素均不是总生存的独立预后因素。结论:手术彻底切除肿瘤降低复发率是治疗FS-DFSP 的关键,肿瘤的复发及转移是影响预后的重要因素。      

Metastatic potential of dermatofibrosarcoma protuberans with fibrosarcomatous change

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon fibrohistiocytic tumor that is locally aggressive. DFSP is associated with frequent local recurrences but rarely metastasizes. It has been suggested that fibrosarcomatous change within a dermatofibrosarcoma protuberans (DFSP-FS) is associated with increased risk of local recurrence and distant metastases. Metastases remain rare, however, and are typically preceded by multiple local recurrences. We report a rare case of a DFSP-FS metastasis to the head of the pancreas without any preceding local recurrence.