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。     

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:     

隆突性皮肤纤维肉瘤51例形态学分析

隆突性皮肤纤维肉瘤（dermatafibroaarcoma protuberans，DFSP）是1种发生于皮肤的结节状或多结节状肿瘤，局部复发率高，现回顾性分析本院收治的51例DFSP，并探讨DFSP形态学特性。     

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32例诊治分析

自１９８０￣１９９４年间收治经病理学证实的隆突性皮肤纤维肉瘤３２例。首次就诊误诊率达７８．１％，７例术前确诊者行局部扩大切除术无复发，２５例误诊者首次手术均行肿瘤单纯切除术，其中８例术后复发。再次发行扩大切除术，随访２￣８年无复发。作者认为提高术前诊断率及首次采用局部扩大切除术是根治该病减少复发的关键。本文结合文献对该病的临床和病理特点、误诊原因及治疗方法进行了讨论。     

乳腺变异型隆突性皮肤纤维肉瘤一例

隆突性皮肤纤维肉瘤（dermatofibrosarcoma protuberans,DFSP）是皮肤真皮组织发生的间叶性肿瘤,是表浅的低度恶性肉瘤。肿瘤的基本特点是位于真皮层,临床表现通常为结节状皮肤肿块,发生于皮下组织而不累及真皮的深部变异型DFSP十分罕见,文献曾报道3例。笔者就本院收治的1例乳腺变异型隆突性皮肤纤维肉瘤,结合文献复习,对发生于乳腺的变异型DFSP的诊断、鉴别诊断及预后进行探讨。     

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。     

放射治疗在隆突性皮肤纤维肉瘤中的地位

目的 研究放射治疗在隆突性皮肤纤维肉瘤（ＤＦＳＰ）中的价值。方法 回顾性分析１９９５年以来收治的１８例ＤＦＳＰ患者,所有病例经组织病理学确诊,手术＋放射治疗１５例,术后放射治疗剂量多数在５０～６０Ｇｙ,其中１例３７．５Ｇｙ后拒绝继续放射治疗,１例因肿瘤残存,照射７０Ｇｙ。单纯放射治疗２例,剂量分别为５２Ｇｙ和５０Ｇｙ。单纯手术１例。结果 ９例既往发生过３１次复发,７／９的第１次复发发生在６个人,本次治疗后,随访０．５～５．０年,中位随访时间２６个月,无瘤生存１６例,失败２例,其中１例单纯放射治疗５２Ｇｙ者,２１个月死于局部未控,１例单纯手术者,４个月后局部复发,手术＋放射治疗的１５例全部达到局部控制。结论 手术切除后放射治疗能有效降低ＤＦＳＰ的复发率。     

隆突性皮肤纤维肉瘤25例临床病理分析

目的探讨隆突性皮肤纤维肉瘤的临床病理特征。方法对25例隆突性皮肤纤维肉瘤的临床表现、组织形态学和免疫组化进行分析。结果全部病例根据临床表现、组织结构、细胞形态及免疫组化确诊。免疫组化示:vim-entin阳性率100%(25/25),CD34阳性率100%(25/25),Ki-67阳性率60%(15/25),SMA阳性率32%(8/25),actin阳性率40%(10/25),CD68、S-100、FⅧa及CK均阴性。结论隆突性皮肤纤维肉瘤容易误诊,需多做切片寻找典型结构和免疫组化进行确诊。     

隆突性皮肤纤维肉瘤25例临床分析

目的探讨隆突性皮肤纤维肉瘤的疗效.方法肿块局部扩大切除,术后3周行60Co-r线+电子束(或深部X线)放疗,观察复发率及3年、5年生存率.结果术后复发率为32%(8/25);3年、5年生存率分别为96%(24/25)、92%(23/25).结论肿块局部扩大切除、术后辅助放疗可有效地降低复发率,对5年无瘤生存,改善患者生存质量及长期生存均有帮助,是隆突性皮肤纤维肉瘤最佳治疗方案.     

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     

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.     

74例隆突型皮肤纤维肉瘤的治疗与预后分析

Objective： To study treatment and prognostic factor in 74 patients with dermatofibrosarcoma protuberans. Methods： From August 1990 to November 1999, 74 patients with dermatofibrosarcoma protuberans （DFSP） confirmed by pathology were treated in Cancer Hospital of Sun Yat-sen University. 72 cases were given wide excision and 2 cases were given local excision. All of 74 cases, 52 cases had surgical resection alone, and 22 cases had surgical resection combined with radiotherapy. Total dose of radiotherapy was 50-70 Gy. Results： The rate of recurrence was 36.1% for all patients. The 5-year recurrence-free survival （RFS） rate was 66%. The 5-year recurrence-free survival rates for resection alone and combined with radiotherapy were 58% and 90%, respectively （P=-0.0187）. The 5-year recurrence-free survival rates for positive microscopic margins and negative microscopic margins were 57% and 75%, respectively （P=0.0468）. Conclusion： Post-operation radiotherapy is an effective treatment to decrease the recurrence rate for the patients with positive microscope, or the patients without suitable surgical treatment.     

隆突性皮肤纤维肉瘤的临床病理及免疫组织化学研究

目的　探讨隆突性皮肤纤维肉瘤的诊断、鉴别诊断、复发原因及其组织起源。方法　对 2 6例隆突性皮肤纤维肉瘤的临床和病理资料进行分析和随访 ,全部标本进行HE、Foot网状纤维染色 ,并采用免疫组织化学ABC法对Vi mentin、AACT、S 10 0、Keratin、NSE和Desmin 6种抗体标记结果进行观察。结果　该肿瘤属低度恶性 ,生长缓慢 ,很少转移 ,但易复发 ;在组织学上具有特征性结构 :瘤细胞围绕胶原纤维或毛细血管作典型的辐射状排列。肿瘤细胞呈梭形、异型性不明显、核分裂少见。免疫组化结果 :2 6例全部对Vimentin呈阳性反应 ,6例对AACT呈阳性反应 ,5例对S -10 0呈阳性反应 ,2 6例全部对Keratin、Desmin和NSE呈阴性反应。结论　隆突性皮肤纤维肉瘤在诊断上 ,主要根据HE染色观察 ,形态上典型 ,结合其临床和病理特征即可确诊 ,对隆突性皮肤纤维肉瘤手术范围不宜过于保守 ,局部要彻底切除干净 ,则可减少其复发机会 ,该肿瘤可能起源于真皮内的具有多种分化潜能的间叶细胞。     

3D-Histological evaluation of surgery in dermatofibrosarcoma protuberans and malignant fibrous histiocytoma: Differences in growth patterns and outcome

Aims

To evaluate the microscopic growth pattern of dermatofibrosarcoma protuberans (DFSP) and malignant fibrous histiocytoma (MFH) and the long-term outcome using 3D-histologic surgery with paraffin sections to cover complete margins and to detect subclinical spreads very sensitively.

Methods

One hundred and one patients have been included comprising 70 DFSP, 31 MFH. Data from 87 patients treated since 1992 were collected prospectively.

Results

Mean clinical tumor-size was 45 mm, mean histological tumor size 65 mm. A mean excision margin of 19 mm achieved negative margins. The histological infiltration shows an asymmetrical pattern with horizontal or vertical extension either cord-, sector- or multiple-like up to 70 mm in length, detectable by 3D-histology. Age and localization differed significantly between DFSP and MFH lesions. MFH tumors had a significantly deeper infiltration than DFSP. The mean follow up was 60 months. In 70 patients with DFSP one local recurrence after 62 months occurred, but no metastasis. 31 patients with MFH developed 8 local recurrences, and 4 metastases (lymph nodes and/ or lungs); 3 of them died of the disease, all 3 had a postoperative status of R1 (p = 0.001).

Conclusions

There are significant differences in growth pattern and clinical outcome between DFSP and MFH. DFSP can be cured by surgery following 3D-histology with paraffin sections. MFH is significantly more malignant. After local R0-resection proofed by 3D-histology higher cure rates can be achieved.     

隆突性皮肤纤维肉瘤21例临床病理分析

目的:探讨隆突性皮肤纤维肉瘤（dermatofibrosarcoma protuberans,DFSP）的临床病理特点及鉴别诊断。方法:对21例DFSP病例标本进行回顾性分析,常规石蜡切片进行HE染色和免疫组化染色,分析其组织学特征及免疫表达。结果:镜下观察,肿瘤由梭形细胞构成,漩涡状排列,免疫组化示21例中所有病例Vimentin、CD34均为阳性,S-100、HMB45部分病例呈阳性表达。结论:DFSP是一种少见的低度恶性肿瘤,形态多种多样,免疫组化检查有助于与其他皮肤纤维组织肿瘤鉴别诊断。     

Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial sarcoma usually affecting the trunk, with significant risk of local recurrence. It is characterized by the presence of ring chromosomes or chromosomal translocations fusing the promoter of the collagen gene COL1A1 to the platelet-derived growth factor beta-chain gene PDGFB, increasing the production of PDGF locally and promoting autocrine or paracrine tumor growth. Fewer than 5% of patients with DFSP develop metastatic sarcoma, with a poor subsequent prognosis. Imatinib (STI-571) was developed as an inhibitor of the PDGF receptor tyrosine kinase and has proven clinical activity against chronic myelogenous leukemia (expressing bcr-abl) and gastrointestinal stromal tumors (expressing c-kit). We describe 2 patients with metastatic and unresectable metastases from DFSP treated with imatinib. After confirmation of negative CD117 status of 2 sarcomas arising from DFSP, patients were given imatinib 400 mg po qd and assessed at regular intervals for their tolerance and response to therapy. One patient had a transient response, then progressed rapidly and died of disease. Another patient showed a partial response to therapy after 2 months, with resolution of superior vena cava syndrome and shrinking of metastatic lung lesions. His response is ongoing after 6 months of therapy. These clinical data confirm findings from models of DFSP and support the use of imatinib in the rare setting of metastatic DFSP. Imatinib may be useful for patients with locally advanced DFSP, when other options for local therapy are limited.     

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.     

外阴隆突性皮肤纤维肉瘤的诊断与治疗

隆突性皮肤纤维肉瘤（dermatofibrosarcoma protuberans, DFSP）为一种生长缓慢的低度恶性纤维肉瘤,具有侵袭性生长、局部复发率高、罕见转移［1］等特点。病变多位于头颈、躯干及四肢等部位,发生于外阴者极为罕见［2］。本文结合国内外文献报道,对我院20年来收治的5例外阴隆突性皮肤纤维肉瘤患者的临床特点及病理学特征加以分析并复习相关文献。