Risk factors related to postoperative recurrence of dermatofibrosarcoma protuberans: A retrospective study and literature review

BACKGROUND Dermatofibrosarcoma protuberans(DFSP)is a rare low-grade malignant soft tissue tumor characterized by rosette-like infiltrative growth.Postoperative recurrence of this tumor is very common.AIM To evaluate the risk factors related to recurrence after wide local excision(WLE)of DFSP and to guide clinical diagnosis and treatment.METHODS The medical records of 44 DFSP patients confirmed by pathology at our hospital from 2012 to 2019 were retrospectively reviewed.The relationship between clinical features,tumor characteristics,treatment,and recurrence risk were analyzed,and the possible risk factors for postoperative tumor recurrence were evaluated.RESULTS There were 44 patients in total,including 21 males and 23 females.The median progression free survival was 36 mo(range,1-240 mo).Twenty patients were treated for the first time,while 24 had previous treatment experience.Forty-two cases were followed for 25.76±22.0 mo,among whom four(9.52%)experienced recurrence after WLE(rate was 9.52%).The recurrence rate in the recurrent group was higher than that in the patients with primary tumor(19.05%vs 0%,P=0.028).Eighteen cases had a history of misdiagnosis(rate was 40.91%).The recurrence rate among patients with previous experience of misdiagnosis was significantly higher than in patients without(68%vs 36.84%,P=0.04).The tumor diameter in patients with a history of treatment was larger than in patients treated for the first time(4.75±0.70 cm vs 2.25±0.36 cm,P=0.004).CONCLUSION To sum up,the clinical manifestations of DFSP are not specific and are easily misdiagnosed,thus commonly causing the recurrence of DFSP.After incomplete resection,the tumor may rapidly grow.Previous recurrence history may be a risk factor for postoperative recurrence,and tumor location may have an indirect effect on postoperative recurrence;however,we found no significant correlation between sex,age,course of the disease,or tumor size and postoperative recurrence.

Dermatofibrosarcoma protuberans: from translocation to targeted therapy

Dermatofibrosarcoma protuberans(DFSP), the most common dermal sarcoma, is a low-grade, slow growing fibroblastic malignant neoplasm that most frequently affects middle aged adults and is characterized by a high local recurrence rate and a low propensity for metastasis. Wide surgical resection or Mohs micrographic surgery(MMS) are the preferred approaches for localized disease, while radiation therapy is warranted for inoperable disease or for cases with positive margins where re-excision is not possible. DFSP is generally regarded as refractory to conventional chemotherapy. Treatment options for systemic disease were limited until the discovery of a unique translocation, t(17;22)(q22;q13)(COL1A1;PDGFB) found in a majority of cases. In recent years, imatinib, a PDGFβR, ABL and KIT inhibitor, has revolutionized systemic therapy in DFSP. In this review, we summarize the epidemiological, clinical, histological and genetic characteristics of DFSP and update the readers on its current management.

The role of radiotherapy in 74 patients with dermatofibrosarcoma protuberans

Objective:To study treatment and prognostic factor in 74 patients with dermatofibrosarcoma protuberans.Meth-ods: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 exci-sion.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.

Dermatofibrosarcoma Protuberans of the Neck: A Case Report

Background: Dermatofibrosarcoma protuberans (DFSP) is rare soft tissue sarcoma but has a locally aggressive nature. Although most cases are of low grade and have a rare metastasis rate, all DFSP variants have a tendency to show local recurrence. Wide excision with negative margins is the treatment of choice. Aim: To report a case of DFSP who presented with an asymptomatic slow growing tumor similar to etiologies such as hypertrophic scars or other benign soft tissue tumors. Case Presentation: A 68-year-old male presented with a large soft tumor located at the left posterior neck. Local excision was done under the preoperative impression of a benign tumor such as lipoma or sebaceous cyst. However the diagnosis of DFSP was made upon histological examination and the patient underwent another surgery to achieve free resection margins under general anesthesia as well as adjuvant radiotherapy. Conclusion: DFSP is a malignant tumor that is diagnosed histopathologically. Due to the low incidence rate, slow-growing nature, and non-alarming initial presentation features, diagnostic delay or even misdiagnosis is not uncommon.

Dermatofibrosarcoma Protuberans—An Atypical Breast Tumor

A 49-year-old woman was referred to the Department of Plastic and Breast Surgery under suspicion of breast cancer after a mammogram revealed a self-discovered tumor in the lower part of her left breast. Clinical examination, mammography, and histopathological examination revealed that the original tumor in the left breast was benign, and an incidental malignant tumor, a dermatofibrosarcoma protuberans (DFSP), was found in the contralateral breast. DFSP is a rare and highly malignant entity that is often silent and difficult to diagnose, making a biopsy essential. Surgical treatment must be aggressive due to the high risk of recurrence, which constitutes a technical challenge. The patient underwent surgery using an oncoplastic approach with a volume-reducing technique to achieve the best possible therapeutic and aesthetic results. Therapeutic breast reduction was performed on the right breast and the tumor was removed within the resected tissue. A contralateral symmetrizing mammoplasty was also performed simultaneously. The patient was discharged without major complications, and no recurrence of the tumor was seen during the 30-month follow-up period. The surgical approach included alternative solutions in addition to conventional lumpectomy or mastectomy. A multidisciplinary, open-minded, and creative approach resulted in a satisfying outcome for this patient.

Dermatofibrosarcoma protuberans of the chest wall： three-dimensional wide excision and reconstruction

Dermatofibrosarcoma protuberans （DFSP） is a poorly differentiated interstitial tumor that originates from the dermis. It occurs predominantly in the 20-50-year- old males. DFSP generally presents as local pink plaques or nodules and subsequently develops into nodular masses accompanied by infiltrative growth to the surrounding tissue, including muscle and bone.

Reverse bilateral latissimus dorsi flap reconstruction after extensive mid back dermatofibrosarcoma protuberans excision:a case report

Surgical resection of soft tissue sarcoma of the trunk can result in large defects requiring complex reconstruction for coverage of vital neurovascular structures and tissue defect. Large defects of the back could be reconstructed with multiple random pattern or local pedicled flaps. We present the case of a 48-year-old patient with a locally advanced dermatofibrosarcoma protuberans of the back. Wide local excision of the lesion was performed. The soft tissue defect measured 22 cm × 20 cm × 4 cm and was reconstructed with bilateral reverse latissimus dorsi myocutaneous (RLDM) flap. Each RLDM flap measured 24 cm × 10 cm. The donor site on the back was closed directly on both sides. The patient recovered well and the two flaps healed uneventfully. Twelve months after surgery the patient is disease-free. The use of a RLDM flap in mid-back reconstructions provided wide well-vascularized soft tissue, minimized risk of infection, and maximized back coverage. This flap is an excellent choice for reconstruction of large defects of the mid-back.

隆突性皮肤纤维肉瘤的分子生物学进展

隆突性皮肤纤维肉瘤(Dermatofibrosarcoma protuberans,DFSP)是最常见的皮肤肉瘤之一,缺乏特异性临床和影像学表现,组织病理学复杂,以缓慢不规则浸润周围组织为生长特点,治疗以手术切除为主。目前,DFSP在临床上存在易误诊、漏诊率高和复发率高的难点,亟待开发出特异性的诊断标志物和有效的治疗靶点。本文将对DFSP的最新分子生物学进展进行综述,以期为该肿瘤的诊断和治疗提供新的思路。

隆突性皮肤纤维肉瘤临床及影像特征分析

目的总结隆突性皮肤纤维肉瘤(DFSP)临床及影像学特征,提高临床诊断水平。方法回顾分析我院32例DFSP患者的体格检查、病理学资料、超声特征(形态及边界、内部回声、血供情况等)、CT特征(密度、有无钙化、坏死出血等)、MRI特征(平扫及增强信号、瘤周水肿、有无弥散受限及深部浸润淋巴结转移等)。结果32例DFSP的平均年龄42.3±6.2(岁),首发/复发(19例/13例),复发年限5至18年,复发区域常为手术瘢痕区,极少远处及淋巴结转移。均表现为CD34和Vimentin阳性或强阳性,Ki67值范围3-20%。常位于躯干和四肢,表现为单发无痛质韧结节或肿块。超声呈不均匀低回声伴内部丰富血流,边界清晰。CT上密度均匀一致,且低于肌肉密度,无钙化坏死出血成分。MRI可见双低信号及“脂肪尾征”、“皮肤尾征”和“筋膜尾征”;轻微瘤周水肿或无水肿;中度至显著强化;DWI呈高信号,ADC值显著减低。结论DFSP的发病率相对较低,易复发易误诊,临床及影像具有一定特征性。

长期误诊的隆突性皮肤纤维肉瘤1例

患者女,61岁,腹部浸润性黯红色斑块20余年,增生1年。皮肤科查体:下腹部可见10 cm×5 cm大小的无痛性黯红色硬结样皮肤增厚,表面凹凸不平,左下方可见数个圆形、椭圆形表面光滑的增生性结节。增生性结节组织病理检查:梭形细胞肿瘤,瘤细胞呈束状或交织状排列,细胞有轻度异形性,核分裂像罕见。免疫组化染色结果:肿瘤细胞CD34(+),Ki-67(10%+)。最终诊断:隆突性皮肤纤维肉瘤。

隆突性皮肤纤维肉瘤一例

隆突性皮肤纤维肉瘤是一种少见的浸润性皮肤软组织肿瘤,具有惰性生长的特点,手术切除后易复发。本文报道隆突性皮肤纤维肉瘤一例,患者,女,47岁,上腹部红色结节1年余,经组织病理和免疫组化确诊,给予手术扩大切除,术后1个月放疗,随访2年余未复发。

超声诊断隆突性皮肤纤维肉瘤

目的探讨隆突性皮肤纤维肉瘤(DFSP)的超声表现。方法回顾性分析12例经手术病理证实的隆突性皮肤纤维肉瘤的分布、超声图像特征及彩色多普勒血流成像特点。结果12例隆突性皮肤纤维肉瘤患者中,11例(91.67%)为单发,直径0.9～8.0cm;分布在躯干66.67(8/12)、四肢近端16.67%(2/12)、头颈部16.67%(2/12);83.33%(10/12)肿瘤出现在躯干及四肢近端的皮肤与皮肤下层。91.67%(11/12)肿瘤呈不均匀弱回声,66.67%(8/12)边界清楚,形态规则,肿瘤内部无液化或钙化且不伴有局部淋巴结转移;91.67%(11/12)肿瘤内彩色多普勒显示有丰富彩色血流信号。结论患者躯干及四肢近端的皮肤与皮下层肿块,边界清楚、形态规则、内部呈不均匀弱回声、内部血流较丰富、无局部淋巴结肿大是隆突性皮肤纤维肉瘤的超声表现。

36例隆突性皮肤纤维肉瘤临床诊疗分析

目的:分析隆突性皮肤纤维肉瘤误诊率、复发率高的原因,探讨提高治愈率的策略。方法:回顾性分析36例隆突性皮肤纤维肉瘤患者的临床和病理资料。全组病例均行肿瘤局部扩大切除术,切缘距离肿瘤缘2.5～3.0cm,术中快速冷冻切片病理检查至边缘和基底阴性。创面行皮片或皮瓣移植修复,3例多次复发患者手术切除同时行125I放射性粒子组织间植入治疗。结果:临床首诊误诊率高达63.8%(23/36)。36例扩大切除后创面一期愈合率95%。中位随访时间3.4(1～6)年,共4例(11.1%)复发,3例辅以125I粒子植入治疗者,随访1～2年均未复发。全组病例未发现远处转移和死亡。结论:隆突性皮肤纤维肉瘤临床症状不典型是误诊率高的主要原因,病理检查是明确诊断的方法,手术扩大切除是提高治愈率的关键,放射性粒子组织间近距离治疗是较好的辅助疗法。

萎缩性隆突性皮肤纤维肉瘤5例临床及组织病理分析

目的:探讨萎缩性隆突性皮肤纤维肉瘤(DFSP)的临床及组织病理特征。方法:对西京皮肤医院确诊的5例萎缩性DFSP进行临床及组织病理学分析。结果:萎缩性DFSP主要表现为缓慢生长的界限清楚的萎缩性斑块,部分皮损可伴有皮下结节或外生性结节。临床上萎缩性DFSP容易误诊,其中4例误诊为硬斑病。萎缩性DFSP组织病理表现为真皮明显变薄,真皮内肿瘤细胞不形成典型的席纹状排列,而是平行于表皮,肿瘤细胞可以不浸润脂肪组织。免疫组化显示肿瘤细胞CD34阳性,CD68、S-100蛋白阴性。结论:相对于经典型DFSP而言,萎缩型DFSP的皮损不呈经典的结节性改变,而是表现为萎缩斑,容易误诊。组织病理表现为真皮萎缩,肿瘤细胞不呈席纹状排列,容易误诊为瘢痕或其他纤维细胞肿瘤。

色素型隆突性皮肤纤维肉瘤8例光镜、免疫组织化学及电镜观察

目的 探讨色素型隆突性皮肤纤维肉瘤 (DFSP)临床病理特征和鉴别诊断。方法 对 8例色素型DFSP进行了光镜和免疫组化染色观察 ,其中 4例做电镜观察。结果 色素型DFSP患者的年龄、性别、肿瘤部位和肿瘤生长方式与普通型DF SP相同。随访结果 3例发生复发 ,无 1例发生转移。 4例肿瘤切面见浅黑色或黑色斑。所有病例既可见典型的DFSP组织形态学特征 ,同时又见散在性分布、数量多少不等的黑色素细胞。本组 6例瘤组织显示普通型DFSP图像 ,2例部分区域显示纤维肉瘤型DFSP图像 ,偶可见血管壁平滑肌增生形成的肌样结节和黏液样变性区。Fontana染色黑色素细胞呈阳性 ,Perls染色则为阴性。梭形瘤细胞呈Vim和CD34阳性 ,CD34阳性具有一定辅助诊断价值 ,S 10 0蛋白和NSE阴性 ;黑色素细胞呈S 10 0蛋白和Vim阳性 ;纤维肉瘤型DFSP区Ki 6 7阳性表达率高于普通型DFSP区。电镜观察 :可见梭形纤维母细胞样细胞和含有许多成熟黑色素小体黑色素细胞。结论 色素型DFSP是一种少见的DFSP亚型 ,临床病理特征与普通型DFSP对比有许多共同之处 ,需与动脉瘤样型纤维组织细胞瘤、弥漫性神经纤维瘤。

皮肤纤维瘤与隆突性皮肤纤维肉瘤的临床病理比较

对皮肤纤维瘤 (DF)与隆突性皮肤纤维肉瘤 (DFSP)在临床、组织形态及免疫组化等方面进行了比较。 DF 20例，平均年龄 37.75岁，好发于四肢，平均直径 0.8cm。瘤细胞呈束状或旋涡状排列，局部可见车辐状结构。平均每 50个高倍视野核分裂为 0.75个。常伴组织细胞和炎细胞浸润、胶原增生、表皮增生及黑色素增多， CD 34(－ )。瘤细胞密度与胶原增生及炎细胞浸润呈负相关，提示 DF可能为反应性病变。 DFSP10例，平均年龄 49.1岁，好发于躯干，平均直径 3.23cm。瘤细胞呈典型的车辐状排列，平均每 50个高倍视野核分裂为 5.5个。肿瘤背景清晰，少数可见炎细胞浸润及表皮增生，无胶原增生及黑色素增多， CD34(＋ )。这些临床及病理特征有助于两者的鉴别诊断。

MRI诊断隆凸性皮肤纤维肉瘤

目的探讨隆凸性皮肤纤维肉瘤（DFSP）的MRI表现。方法收集经病理证实的8例DFSP患者,回顾性分析其MRI表现。结果 8例DFSP中,病灶位于躯干3例,四肢5例,均位于皮肤及皮肤下层,肿瘤最大径1.8~11.6 cm。T1WI示8例肿瘤呈等或略低于肌肉信号,T2WI示肿瘤呈明亮高信号,2例病灶内部见斑点状低信号;脂肪抑制（STIR）序列示6例肿瘤呈高信号;1例对比剂动力学时间分辨成像（TRICKS）动态增强MRA示肿瘤的供血血管为腓动脉。6例接受增强扫描者病灶均明显强化,2例病灶内见斑点状无强化出血区。结论 DFSP好发于四肢、躯干及头颈部皮肤及皮下组织,其MRI表现具有一定特征性。

隆突性皮肤纤维肉瘤诊断与治疗

目的：探讨隆突性皮肤纤维肉瘤（DFSP）的诊断和治疗方法。方法：对28例经病理证实不同部位隆突性皮肤纤维肉瘤患者的诊治情况进行回顾性分析。躯干部16例（57％），四肢部8例（28．5％），头颈部4例（14．2）。5例行局部切除术，23例扩大切除术＋植皮或皮瓣转移修复术。16例在术后行放射治疗，平均照射剂量60Gy（50～70Gy）。结果：切除28例标本，CD34阳性26例，阴性2例。术后随访1～5年（平均2．3年），失访2例。复发9例，平均复发时间2．6年（8个月～5年）。28例均未见有远处转移，无死亡。结论：隆突性皮肤纤维肉瘤是一种低度恶性肿瘤，行扩大切除术＋辅助放疗是治疗隆突性皮肤纤维肉瘤的主要治疗方法，手术范围大小及术后辅助放疗与否是影响隆突性皮肤纤维肉瘤的独立预后因素，可有效提高患者生存率及生存质量。

隆突性皮肤纤维肉瘤免疫表型和COL1A1/PDGFB融合基因的临床应用研究

目的:探讨隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)诊断中免疫表型和荧光原位杂交(fluorescence in situ hybridization,FISH)检测COL1A1/PDGFB融合基因的应用价值。方法:观察73例DFSP中免疫组织化学标记物vimentin、CD34、CD99、S100、desmin、SMA和FISH检测COL1A1/PDGFB融合基因的表达。选取85例非DFSP作为免疫组织化学的对照组,10例非DFSP作为FISH检测COL1A1/PDGFB融合基因的对照组。结果:vimentin、CD34、CD99、S100、desmin、SMA在73例DFSP中阳性率分别是100%、91.78%、61.64%、0、0、6.85%,在对照组中不同程度表达,其中CD34的表达在鉴别诊断中有意义。COL1A1/PDGFB融合基因在DFSP的阳性率为86.96%(60/69),对照组均阴性。结论:在DFSP的诊断中,COL1A1/PDGFB融合基因是DFSP较为特异性、敏感性的标记,而CD34是DFSP相对理想的标记。

隆突性皮肤纤维肉瘤的CT及MRI表现

目的探讨隆突性皮肤纤维肉瘤(DFSP)的CT及MRI征象。方法回顾分析16例经手术病理结果证实的DFSP的影像资料(11例接受CT检查,5例接受MR检查),分析肿瘤的形态、CT及MRI特征。结果 DFSP多发于躯干及头颈部皮肤,呈不同程度隆起于皮肤表面,部分悬吊于皮外。16例中,结节肿块型12例、弥漫型4例。CT上肿块呈与肌肉等密度或稍低密度的实性占位,MRI上T1WI呈等/低信号,T2WI为稍高信号。肿瘤血供较丰富,增强扫描多呈渐进性中度至明显强化。病灶内征象包括"悬吊征"2例,"皮肤尾征"6例,"筋膜尾征"1例,"脂肪尾征"4例。结论 DFSP多表现为皮肤及皮下结节肿块型或弥漫生长型实性病变,不同形态其征象不同,但仍具有一定的特征性。