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Dermatofibrosarcoma Protuberans of the Breast: A Rare Localization
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作者 Foba Mamadou Lassana Bathily Yaye Coumba +1 位作者 Teuw El Hadj Daour Sankale Anne Aurore 《Advances in Breast Cancer Research》 CAS 2024年第3期36-42,共7页
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 p... 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. 展开更多
关键词 BREAST dermatofibrosarcoma SURGERY
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Darier Ferrand Mammary Dermatofibrosarcoma Simulating a Breast-Type Myofibroblastoma: A Case Report
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作者 Michel Auguste Mouelle Sarah Gaëlle Adiang Esther Meka 《Advances in Breast Cancer Research》 CAS 2023年第1期10-16,共7页
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-o... 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. 展开更多
关键词 MYOFIBROBLASTOMA dermatofibrosarcoma of Darier Ferrand BREAST
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Dermatofibrosarcoma Protuberans—An Atypical Breast Tumor
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作者 Bu Youn Cho Michael Munksdorf Michael Rose 《Case Reports in Clinical Medicine》 2023年第6期196-201,共6页
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 exami... 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. 展开更多
关键词 dermatofibrosarcoma Protuberans Breast Tumor Oncoplastic Breast Surgery
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Risk factors related to postoperative recurrence of dermatofibrosarcoma protuberans: A retrospective study and literature review 被引量:3
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作者 Jian-Xia Xiong Tao Cai +4 位作者 Li Hu Xiao-Li Chen Kun Huang Ai-Jun Chen Ping Wang 《World Journal of Clinical Cases》 SCIE 2021年第20期5442-5452,共11页
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 t... 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. 展开更多
关键词 dermatofibrosarcoma protuberans RECURRENCE Clinical features Wide local excision SURGERY Retrospective research
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Dermatofibrosarcoma protuberans: from translocation to targeted therapy 被引量:6
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作者 Jonathan Noujaim Khin Thway +1 位作者 Cyril Fisher Robin L.Jones 《Cancer Biology & Medicine》 SCIE CAS CSCD 2015年第4期375-384,共10页
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 ... 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. 展开更多
关键词 dermatofibrosarcoma protuberans(DFSP) imatinib Mohs micrographic surgery(MMS) translocation targeted therapy
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The role of radiotherapy in 74 patients with dermatofibrosarcoma protuberans
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作者 Xiushen Wang Mengzhong Liu +1 位作者 Hui Liu Nianji Cui 《The Chinese-German Journal of Clinical Oncology》 CAS 2006年第6期454-457,共4页
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 path... 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. 展开更多
关键词 dermatofibrosarcoma protuberans (DFSP) RADIOTHERAPY wide excision
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Recurrent Dermatofibrosarcoma Protuberant of the Hand: A Case Report
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作者 Toky Rakotoarivo Malala Razakanaivo +3 位作者 Norosoa Randriamaroson Clairette Raharisolo Jean Claude Razafimahandry Florine Rafaramino 《Journal of Cancer Therapy》 CAS 2022年第7期425-429,共5页
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 i... 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. 展开更多
关键词 dermatofibrosarcoma HAND Wide Surgery RECURRENCE
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Dermatofibrosarcoma Protuberans of the Neck: A Case Report
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作者 Tzu-I Wu Hsing-Mei Wu +2 位作者 Yih-Jeng Tsai Tzu Hsuan Luo Chia-Jung Lee 《International Journal of Otolaryngology and Head & Neck Surgery》 2021年第2期85-91,共7页
<strong>Background:</strong> 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, al... <strong>Background:</strong> 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. <strong>Aim:</strong> 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. <strong>Case Presentation:</strong> 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. <strong>Conclusion:</strong> 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. 展开更多
关键词 dermatofibrosarcoma Protuberans Soft Tissue Sarcoma SURGERY
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隆突性皮肤纤维肉瘤的分子生物学进展
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作者 邓雨琦 梁筱 +2 位作者 孙笛 刘菲 杨军 《组织工程与重建外科》 CAS 2024年第2期260-264,共5页
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... 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。 展开更多
关键词 隆突性皮肤纤维肉瘤 分子生物学 重排 变异
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外阴隆突性皮肤纤维肉瘤一例
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作者 刘思敏 王佳丽 +2 位作者 张世霞 魏佳 杨永秀 《国际生殖健康/计划生育杂志》 CAS 2024年第6期490-493,共4页
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... 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。 展开更多
关键词 外阴肿瘤 皮肤纤维肉瘤 诊断 治疗 预后 病例报告
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隆突性皮肤纤维肉瘤的影像表现及临床病理特征
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作者 熊瑶 尚柳彤 +3 位作者 李文华 段红莉 周中华 李天然 《放射学实践》 CSCD 北大核心 2024年第1期108-114,共7页
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... 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。 展开更多
关键词 皮肤纤维肉瘤 体层摄影术 X线计算机 磁共振成像 病理学
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隆突性皮肤纤维肉瘤临床及影像特征分析
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作者 肖阳 薛影 梁寿衡 《中国CT和MRI杂志》 2024年第11期174-176,共3页
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... 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。 展开更多
关键词 隆突性皮肤纤维肉瘤 超声 计算机断层扫描 磁共振成像
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隆突性皮肤纤维肉瘤一例
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作者 俞顺星 张诗喻 +2 位作者 林越 闫志康 何威 《中国麻风皮肤病杂志》 2024年第7期506-507,共2页
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... 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。 展开更多
关键词 隆突性皮肤纤维肉瘤
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Dermatofibrosarcoma protuberans of the chest wall: three-dimensional wide excision and reconstruction
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作者 Zhang Zhenyu Cen Ying Qing Yong Wang Huaisheng Liu Xiaoxue 《Chinese Medical Journal》 SCIE CAS CSCD 2014年第2期386-388,共3页
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 plaqu... 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. 展开更多
关键词 dermatofibrosarcoma protuberans SURGERY chest wall RECONSTRUCTION
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Reverse bilateral latissimus dorsi flap reconstruction after extensive mid back dermatofibrosarcoma protuberans excision:a case report
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作者 Stefano Bonomi Laura Sala +2 位作者 Alessandro Gronchi Dario Callegaro Umberto Cortinovis 《Plastic and Aesthetic Research》 2018年第3期1-8,共8页
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 ... 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. 展开更多
关键词 REVERSE latissimus dorsi myocutaneous flap TRUNK reconstruction posterior TRUNK defect SARCOMA dermatofibrosarcoma protuberans
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乳腺隆突性皮肤纤维肉瘤21例临床病理及分子学特征 被引量:2
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作者 黄海建 谢飞来 +2 位作者 李柏成 郑松龄 陈小岩 《临床与实验病理学杂志》 CAS 北大核心 2023年第7期788-792,共5页
目的探讨乳腺隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans of the breast,DFSP-B)的临床病理学及分子学特征。方法回顾性分析21例DFSP-B的临床病理学、免疫表型及分子学特征,行HE、免疫组化及分子检测,并复习相关文献。结果21... 目的探讨乳腺隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans of the breast,DFSP-B)的临床病理学及分子学特征。方法回顾性分析21例DFSP-B的临床病理学、免疫表型及分子学特征,行HE、免疫组化及分子检测,并复习相关文献。结果21例DFSP-B患者男性6例,女性15例,年龄22~75岁(平均39.76岁,中位年龄36岁)。肿瘤最大径1~15 cm(平均4.7 cm,中位3 cm)。镜下瘤细胞呈席纹状、漩涡状及束状排列,核分裂象2~15个/10 HPF,侵犯脂肪及乳腺组织,符合DFSP-B;其中经典型DFSP-B 15例,经典型伴巨细胞纤维母细胞瘤(giant cell fibroblastoma,GCF)1例,黏液型2例,纤维肉瘤型隆突性皮肤纤维肉瘤(fibrosarcomatous-dermatofibrosarcoma protuberans,FS-DFSP)2例,FS-DFSP伴黏液型1例。免疫表型:CD34(21/21)、H3K27Me3(21/21)、β-catenin(21/21,胞膜)和WT-1(4/8,胞质)均阳性,Ki-67增殖指数为5%~50%。FISH检测DFSP-B中PDGFB基因分离阳性(7/8);3例FS-DFSP中PDGFB基因分离阳性(1/1),COL1A1-PDGFB融合阳性(1/1)。2例行NGS检测,其中1例FS-DFSP检测到CDKN2A基因(p.L16fs)突变及MSH6基因(p.F1088fs)突变;1例经典型伴GCF型检测到MLH1基因(p.R487)突变及TP53基因p.R273H体系突变。结论DFSP-B诊断需结合临床病理、免疫表型及PDGFB基因检测,治疗以手术切除为主,必要时可行放疗及靶向治疗,预后较好。 展开更多
关键词 乳腺肿瘤 隆突性皮肤纤维肉瘤 PDGFB基因 免疫组织化学
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隆突性皮肤纤维肉瘤36例临床分析
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作者 李艳 杨子良 +1 位作者 宋琳毅 周乃慧 《临床皮肤科杂志》 CAS CSCD 北大核心 2023年第3期131-134,共4页
目的:总结隆突性皮肤纤维肉瘤(DFSP)的临床表现、影像学表现及组织病理特点。方法:回顾性分析1988年9月-2020年12月就诊于苏州大学附属第一医院的36例DFSP患者的临床及组织病理资料。结果:36例DFSP患者中,男女比例1.6:1;平均发病年龄(36... 目的:总结隆突性皮肤纤维肉瘤(DFSP)的临床表现、影像学表现及组织病理特点。方法:回顾性分析1988年9月-2020年12月就诊于苏州大学附属第一医院的36例DFSP患者的临床及组织病理资料。结果:36例DFSP患者中,男女比例1.6:1;平均发病年龄(36.8±11.4)岁。皮损以躯干最为多见[25例(69.4%)],其次为四肢[7例(19.4%)]。超声检查示肿瘤位于真皮及皮下层,呈不均匀低回声,边界清楚,肿瘤内具有较丰富的血流信号。磁共振成像(MRI)平扫示T1WI呈等或低信号,T2WI呈高信号,增强后可见明显均匀强化。36例患者组织病理分型均为经典型,临床诊断与病理诊断符合率为16.7%,免疫组化示CD34和vimentin表达阳性,S-100蛋白和CD68表达阴性。该组患者术后复发率为27.6%。结论:DFSP多见于中年男性,病程缓慢,临床误诊率高。超声及MRI等影像学检查可为疾病的诊断、手术方式及范围提供参考。该病复发率高,手术应局部扩大切除,尤其需注意切除的范围和深度,加强长期临床随访。 展开更多
关键词 隆突性皮肤纤维肉瘤 临床 组织病理
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Darier-Ferrand Dermofibrosarcoma: A Case Report of a Cervical Localization
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作者 Aminata Mbaye Ndiassé Ndiaye +5 位作者 Ndeye Fatou Thiam Ousmane Sano Rachid Vitamine Mouhamed Salem Ould Abderrahmane Maïmouna Diagne Malick Ndiaye 《International Journal of Otolaryngology and Head & Neck Surgery》 2023年第5期317-325,共9页
Darier-Ferrand dermatofibrosarcoma (DFS) is a cutaneous mesenchymal tumor of intermediate malignancy. It is a rare but not exceptional tumor, accounting for 0.1% of malignant skin tumors. Histological examination is e... Darier-Ferrand dermatofibrosarcoma (DFS) is a cutaneous mesenchymal tumor of intermediate malignancy. It is a rare but not exceptional tumor, accounting for 0.1% of malignant skin tumors. Histological examination is essential for diagnosis. Wide surgical excision is the standard treatment. DFS is a tumor whose prognosis and evolutionary risk are mainly linked to the delay in diagnosis and the quality of the first excision. Late diagnosis makes excision and reconstruction surgery difficult. The chances of recovery in the case of well-performed primary surgery are significantly greater than in the case of salvage surgery. To improve prognosis, early, codified, multidisciplinary management is essential. In our African context, and especially in the case of patients living in rural areas, errant diagnoses are often found hence the importance of raising awareness and providing information to healthcare personnel. We report a case of an enlarged left supraclavicular Darier-Ferrand dermatofibrosarcoma. The patient had come for a late consultation at a stage when the tumour was large. The CT scan was a great help in the pre-operative phase. The patient underwent complete surgical excision, the postoperative course was straightforward and the histology of the surgical specimen confirmed the diagnosis. The resection margins were healthy. The patient is alive at one year with no recurrence or metastasis. 展开更多
关键词 Cervical Localization dermatofibrosarcoma Skin Tumor Wide Surgery
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隆突性皮肤纤维肉瘤55例临床病理分析 被引量:1
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作者 顾婷婷 姚丽倩 +2 位作者 王雨潇 徐松 高小姣 《南通大学学报(医学版)》 2023年第4期346-350,共5页
目的:分析55例隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)患者的临床表现和病理,讨论其诊断和鉴别诊断要点,提高对该肿瘤的认识。方法:回顾性分析昆山地区2007年1月—2021年1月期间诊断的55例DFSP患者的临床表现、病理... 目的:分析55例隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)患者的临床表现和病理,讨论其诊断和鉴别诊断要点,提高对该肿瘤的认识。方法:回顾性分析昆山地区2007年1月—2021年1月期间诊断的55例DFSP患者的临床表现、病理组织学特点、治疗及预后。结果:DFSP临床多表现皮肤瘢痕或萎缩性斑块结节,表面可有破溃,肿块直径平均(3.31±1.04)cm。发病部位主要为躯干、头颈肩部,也可发生于乳房、腹股沟等少见部位。病变多位于真皮层,单发结节,偶为多发,切面呈褐色、粉红色、灰白色,与周围组织界限相对较清,可浸润皮下脂肪及横纹肌。组织形态大多为梭形、短梭形,可呈轮辐状、漩涡状或花边样排列,可伴有黏液变性,间质硬化,以及出现色素细胞分布。免疫组织化学染色显示肿瘤细胞主要表达CD34和Vimentin,也可在胞质表达BCL-2和β-Catenin。4例行FISH荧光原位杂交分子检测,结果均存在血小板衍生的生长因子B链基因重排。55例DFSP中行局部扩大切除术14例,行局部切除术41例。随访的30例患者中6例术后局部复发。结论:DFSP是易发生于真皮的低度恶性软组织肿瘤,掌握DFSP的临床病理特点,通过免疫组织化学染色标记及分子检测能有效地诊断,避免与其他皮肤梭形细胞肿瘤混淆;主要的治疗方式是外科手术扩大切除,局部切除易复发。 展开更多
关键词 隆突性皮肤纤维肉瘤 免疫组织化学染色 FISH 血小板衍生的生长因子B链基因重排 临床病理
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影像学术前评估联合改良慢Mohs显微描记手术治疗隆突性皮肤纤维肉瘤一例
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作者 谢凯 张晓俊 +5 位作者 郭璇 耿建辉 王金良 李建可 陈声利 刘国艳 《中国麻风皮肤病杂志》 2023年第12期884-886,共3页
患者,男,33岁。右侧前胸壁浸润性斑块10年,增生1年。组织病理诊断隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)。术前予以行强化MRI判断肿瘤侵袭深度,高频超声辅助判断肿瘤边缘,两种影像学检查联合完成术前评估。排除手... 患者,男,33岁。右侧前胸壁浸润性斑块10年,增生1年。组织病理诊断隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)。术前予以行强化MRI判断肿瘤侵袭深度,高频超声辅助判断肿瘤边缘,两种影像学检查联合完成术前评估。排除手术禁忌后予以行改良慢Mohs显微描记手术(slow modified Mohs micrographic surgery,smMMS)切除肿瘤,中厚皮植皮修复创面,术后恢复可,无术后并发症。 展开更多
关键词 隆突性皮肤纤维肉瘤 Mohs显微描记手术
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