An unusual dermatofibrosarcoma protuberans: a case report

Dermatofibrosarcoma protuberans (DFSP) previously called Morbus Darier is an intermediate grade sarcoma with a fairly high rate of recurrence, it occurs most commonly on the trunk and extremities but may also occur in the head and neck region. Treatment of choice is wide excision. A 69-year-old female patient with a 57-year history of dermatofibrosarcoma protuberans is presented. The lesion appeared on the right forehead and both eyelids at the age of 12 years. The patient required 64 surgeries mostly for the head and neck lesions.     

伴有肌样/肌纤维母细胞性分化的隆突性皮纤维肉瘤的临床病理分析

目的 探讨隆突性皮纤维肉瘤（ＤＦＳＰ）中肌样／肌纤维母细胞性分化的本质及其临床病理学意义。方法 采用常规ＨＥ切片对１２４例ＤＦＳＰ进行筛选,对6例伴有肌样／肌纤维母细胞性分化的ＤＦＳＰ病例进行免疫组织化学标记,其中２例加做电镜检测。结果 肌样／肌纤维母细胞性分化多出现在纤维肉瘤型ＤＦＳＰ（ＦＳ－ＤＦＳＰ）中,表现为肿瘤周边部或肿瘤内散在性分布的深嗜伊红色小结节或短要束,由梭形细胞组成,细胞多无异型性,核分裂象也罕见,形态上似平滑肌细胞或肌纤维母细胞。免疫组织化学标记显示肌样区域细胞表达α－平滑肌肌动蛋白和肌物质特异性肌动抗原,不表达ＣＤ３４;电镜观察证实细胞含有质膜下微丝束、局灶性致密体及微胸饮囊泡样结构,与肌纤维母细胞相一致,结论 ＤＦＳＰ中的肌样／肌纤维母细胞性分化可能是间质中肌纤维母细胞增生的结果,并非代表了瘤细胞的真性肌纤维母细胞性分化。     

A case of dermatofibrosarcoma protuberans in the skin over the breast

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing but locally aggressive, fibrous tumor that has a high rate of local recurrence after surgical resection. This tumor occurs most commonly in the trunk and proximal extremities. In this report we present a case of dermatofibrosarcoma protuberans in the skin over the breast which was re-excised after pathological diagnosis, considering cosmetic aspects. Only three other cases of dermatofibrosarcoma protuberans of the anterior chest wall have been encountered in our hospital, and all of these cases were male. The presence of this tumor in the skin over the breast appears to be rare in females. Careful complete resection is recommended for this type of tumor.     

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.     

Molecular and clinicopathological analysis of dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a dermal and subcutaneous tumor of intermediate malignancy. The most remarkable cytogenetic feature of DFSP is the chromosomal translocation t(17;22)(q22;q13), causing a fusion of the platelet-derived growth factor beta chain (PDGFB) gene at 22q13, and the collagen type 1 alpha 1 (COL1A1) at 17q22. The aim of the study was to analyze the molecular characteristic of DFSP in conjunction with histopathological and clinical features.     

隆突性皮肤纤维肉瘤的MRI和CT表现

目的探讨隆突性皮肤纤维肉瘤（DFSP）的MRI与CT特征。方法回顾性分析11例经手术及病理证实的隆突性皮肤纤维肉瘤患者的MRI及CT资料。11例中,8例接受MR检查,其中5例接受增强扫描;4例接受CT扫描,其中1例同时接受增强扫描。结果10例DFSP位于皮下及皮下脂肪层,表现为边界清楚的椭圆形或分叶状肿块;1例位于左侧大腿中上段深部。DFSP在T1WI图像上主要为低至稍高信号,T2WI图像上呈稍高或高信号;CT平扫呈等密度。MR和CT增强扫描病灶均明昂强化。结论隆突件皮肤纤维肉瘤的MRI和CT嘉孤且右一宦特衍．右助千明确诊断．     

The fibroblastic nature of dermatofibrosarcoma protuberans

Summary Dermatofibrosarcoma protuberans has been considered to be of fibrohistiocytic or fibroblastic origin. The purpose of this paper is to identify the original cell strain from which this neoplasm derives, using tissue culture and electron microscopic methods. Thirteen cases of DFSP characterised by clinical, topographical, histological and behavioral criteria were explanted. The emigrating cells were bipolar with two opposed processed and showed a radial arrangement in respect to the expiants. After the second week the distal processes tended to curve back towards the cell body forming flame-like structures. This cell morphology and cellular orientation persisted during the whole life of the culture. Electron microscopy was performed in three cases; the newly grown cells maintained an electron microscopic picture similar to that found in the original tumors.This pattern of behaviour is characteristic of fibroblastic tumors and has been found in explants of normal fibroblasts, of fibromatosis and of fibrosarcomas used as controls. On this basis, we believe that DFSP is a fibrosarcoma of the skin of low grade malignancy.     

Dermatofibrosarcoma protuberans of the head and neck: treatment with Mohs surgery using inverted horizontal paraffin sections

OBJECTIVE: To describe results of treating dermatofibrosarcoma protuberans of the head and neck with a Mohs surgical technique in which tumor margins were evaluated with inverted horizontal paraffin sections to identify the margin of resection necessary to achieve tumor-free borders. STUDY DESIGN: Retrospective case study of nine patients. METHODS: In each case, Mohs surgery using inverted horizontal frozen-section analysis was continued until tumor-free margins were achieved. The tumor-free specimens collected during this procedure were immediately reprocessed by using inverted horizontal paraffin sectioning, and the histological results of the two techniques were compared. To identify the margins of resection needed to achieve tumor-free margins, preoperative lesion size was compared with postoperative defect size. RESULTS: Paraffin section analysis showed persistent tumor in seven of the nine patients in whom the frozen-section technique was used to clear the tumor. Tumor-free margins were achieved in all patients by using one additional excision. The tumor had spread extensively beyond clinical margins and required a mean minimum margin of 3.7 cm around the gross area of tumor (margin range, 2.5-6.0 cm). Patients treated by this technique showed no local, regional, or distant recurrence at follow-up performed at a mean time of 43 months postoperatively (range, 19-74 mo). CONCLUSIONS: Dermatofibrosarcoma protuberans of the head and neck is a tumor that shows extensive infiltration beyond gross margins. Wide local excision with 2- to 3-cm margins results in an unacceptably high recurrence rate; larger excisional margins are necessary to remove all disease. Accurate margin identification achieved by using Mohs surgery with rush paraffin section analysis results in complete tumor removal and excellent control rates. This method should be preferred for treatment of dermatofibrosarcoma protuberans of the head and neck.