Dermatofibrosarcoma Protuberans of the Breast: A Rare Localization

The dermatofibrosarcoma protuberans (DFP) is a rare skin tumor. It represents 0.1% of the malignant skin tumor. Surgery is its only treatment. The breast involvement is exceptional. We report our cases, 13 years old patient we have dermatofibrosarcoma protuberans in her breast.

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.

Recurrent Dermatofibrosarcoma Protuberant of the Hand: A Case Report

Dermatofibrosarcoma is a rare, slow growing tumor with a tendency to local recurrence. The treatment is mainly surgical. In Madagascar there is no specialized center for the management of soft tissue sarcomas. It is in this context that we report a case of a 45-year-old man, driver, presenting with recurrent dermatofibrosarcoma of the left hand. The diagnosis was confirmed by the positivity of the CD34 marker. Extensive local excision surgery was performed with the patient’s consent. After free years of treatment, there is no tumor recurrence.

Darier Ferrand Mammary Dermatofibrosarcoma Simulating a Breast-Type Myofibroblastoma: A Case Report

Myofibroblastoma and Darier Ferrand’s dermatofibrosarcoma are rare entities that are similar both in terms of clinical morphological characteristics and histological characteristics. We report the case of a 49-year-old non-menopausal woman with a history of right breast lumpectomy. Supported by the Chompret criteria, an oncogenetic consultation was performed. Clinical examination revealed a firm 25 mm mass on the medial part of the left breast with skin involvement. A biopsy was performed and analysis result came back in favor of a cellular type myofibroblastoma showing a fibrous component consisting of spindle cells with a herringbone arrangement. Anatomopathological results concluded to a dermatofibrosarcoma of Darier Ferrand while immunohistochemistry stated a tumor population strongly positive for CD34 expression. The search for a rearrangement of the collagen type I alpha 1 gene (COL1A1) by fluorescence in situ hybridization (FISH) was positive. The diagnosis of Dermatofibrosarcoma of Darier Ferrand can be suspected on imaging and confirmed by histology. Surgical treatment of Darier Ferrand dermatofibrosarcoma consists of wide excision of the lesions with margins greater than 2 cm, on which the prognosis mainly depends. Micrographic surgery and oncoplastic breast surgery are of major interest in this location.

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。

外阴隆突性皮肤纤维肉瘤一例

隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)是一种少见的真皮间质肿瘤,而发生于外阴的DFSP在临床上更为罕见,病理学检查结合免疫组织化学染色是明确诊断的主要方法,常以手术治疗为主。本文报告1例外阴DFSP,该患者既往于当地医院行外阴肿物切除术,术后病理检查提示DFSP,遂转往上级医院行补充治疗。经手术切除残余病灶后送病理及免疫组织化学检查,再次明确诊断为外阴DFSP,术后给予补充化疗。通过探讨该病的病因及发病机制、临床表现、病理分型、诊断、鉴别诊断、治疗、预后及随访情况,以加深临床对该病的认识。

隆突性皮肤纤维肉瘤的影像表现及临床病理特征

目的:探讨隆突性皮肤纤维肉瘤(DFSP)的影像表现及临床病理特征,提高该病的诊断及鉴别水平。方法:回顾性分析经病理证实的35例DFSP患者的临床及影像资料。结果:35例患者中单发31例,多发4例,共40个病灶;34例位于皮肤或皮下脂肪层,1例位于浅筋膜下层;发生于躯干21例,四肢10例,头面部4例。病灶多呈不规则形或梭形,其中14个病灶伴有“子结节外突征”,23个病灶伴有“多结节征”,5个病灶伴有“悬吊征”。病灶最大径1.3~18.6 cm,平均5.1 cm。病变CT平扫呈等或稍低密度,增强扫描多呈轻中度不均匀强化。T1WI多呈等、稍低信号,T2WI-FS多呈均匀或混杂高信号,部分病灶内可见“双低信号征”,DWI呈高信号,增强扫描呈均匀或不均匀明显强化。病灶多合并周围侵袭征象,26例见“皮肤尾征”,32例见“脂肪尾征”,16例见“筋膜尾征”。病理表现为真皮及皮下组织的梭形肿瘤细胞围绕血管呈“车辐状”排列。免疫组化CD34、Vimentin多呈阳性,CK、S-100蛋白多呈阴性。结论:DFSP的发病部位、CT及MRI表现具有一定特征性,术前检查有助于其准确定性及临床手术方案制定。

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。

隆突性皮肤纤维肉瘤72例临床病理学观察

目的 观察隆突性皮肤纤维肉瘤 (DFSP)的临床病理特点 ,探讨诊断、鉴别诊断及其组织起源。方法 对 72例DFSP病例进行临床表现、组织形态学、免疫组化研究 ,16例真皮纤维瘤 (DF)、19例神经纤维瘤 (NF)、17例纤维肉瘤 (FS)分别作为对照。结果 72例DFSP好发中青年男性 ,肿瘤为单发或多发性结节 ,位于真皮 ,可浸润皮下脂肪及横纹肌。组织形态除经典的车辐状或席纹状结构外 ,还存在一些变异如黏液变性、伴FS区域、Bednar瘤等。DFSP组 87%CD34阳性 ,NF组 4 2 %CD34阳性。结论 掌握DFSP的临床病理特点 ,避免与其它皮肤梭形细胞肿瘤尤其是DF、NF及黏液性肿瘤混淆。

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。

肌样型隆突性皮肤纤维肉瘤中肌样区域的组织发生和性质

目的 探讨肌样型隆突性皮肤纤维肉瘤 (DFSP)中肌样区域的组织发生和性质。方法 对 95例DFSP中筛选出的15例肌样型DFSP进行光镜和免疫组织化学研究。结果 临床资料显示女性比男性稍多见 (1 5∶1)。 18～ 4 0岁为发病高峰年龄段 (73% )。肿瘤部位常见于躯干和肢体 (11/15 )。镜检 :在 15例肌样型DFSP中 (9例为纤维肉瘤型 ,最为常见 ;4例为经典型 ;2例为色素型 ,后者伴有肌样区域未见文献报道 )均观察到散在分布的肌样结节和肌样束 ,即肌样区域 ,且与瘤内血管壁平滑肌细胞增生有密切的关系 ,并发现血管壁改变具有不同程期的特点。早期 :瘤内小血管和少许较大血管壁平滑肌细胞显著增生 ,细胞无异型性 ,可见核分裂象 ;中期 :增生的平滑肌细胞形成特征性的嗜伊红性肌样结节和肌样束 ,可伴轻度玻璃样变 ,在大多数肌样结节和肌样束中常可见偏位、不规则、变小变窄的血管腔 ;后期 :肌样结节和肌样束可相互融合 ,血管腔萎陷或消失 ,伴有广泛玻璃样变甚至钙化。免疫表型 :肌样结节和肌样束呈SMA、MSA、Vim弥漫强阳性 ,但对Des、smoothmusclemyosin、caldesmon和CD34均呈阴性。需特别指出的是肌样结节和肌样束中偏位的血管腔衬覆的内皮细胞呈CD34阳性 ,证实了肌样区域与血管壁的密切关系。