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Recurrent dermatofibrosarcoma protuberans involving the lacrimal sac:A case report 被引量:1
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作者 Bijnya Birajita Panda Sudhakar Gunasekar +2 位作者 Utkarsh Agarwal Thilakraj Koppalu Lingaraju Amit Kumar Adhya 《World Journal of Clinical Cases》 2025年第30期112-118,共7页
BACKGROUND Dermatofibrosarcoma protuberans(DFSP)is a rare,low-grade,locally aggressive cutaneous sarcoma.DFSP in the periocular region is exceedingly rare,leading to diagnostic and surgical challenges due to anatomica... BACKGROUND Dermatofibrosarcoma protuberans(DFSP)is a rare,low-grade,locally aggressive cutaneous sarcoma.DFSP in the periocular region is exceedingly rare,leading to diagnostic and surgical challenges due to anatomical constraints in the periocular region.Precise diagnosis is essential to guide appropriate surgical management and prevent recurrence.CASE SUMMARY A 32-year-old female presented with a recurrent tumor in the medial canthus,previously diagnosed as a solitary fibrous tumor in an outside institution.After complete radiological and systemic workup,she was scheduled for a wide local excision followed by reconstruction after getting tumor clear margins on frozen section.Histopathology confirmed DFSP,characterized by storiform spindle cell proliferation,diffuse cluster of differentiation 34 positivity,and signal transducer and activator of transcription 6 negativity.CONCLUSION This case highlights the challenges in the diagnostic and surgical management of DFSP in periocular tumors.Comprehensive surgical excision with appropriate reconstruction is critical for achieving oncological control while preserving aesthetics and function. 展开更多
关键词 dermatofibrosarcoma protuberans Medial canthus tumor Solitary fibrous tumor Wide local excision Cluster of differentiation 34 Signal transducer and activator
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Beyond COL1A1::PDGFB:Rare fusions and their clinical implications in dermatofibrosarcoma protuberans
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作者 Sumanta Das Sunita Ahlawat 《World Journal of Clinical Cases》 2025年第33期119-125,共7页
Dermatofibrosarcoma protuberans(DFSP)is a rare cutaneous intermediate-grade soft tissue tumor characterized by COL1A1::PDGFB fusion in most cases.This fusion drives tumorigenesis and forms the basis for imatinib treat... Dermatofibrosarcoma protuberans(DFSP)is a rare cutaneous intermediate-grade soft tissue tumor characterized by COL1A1::PDGFB fusion in most cases.This fusion drives tumorigenesis and forms the basis for imatinib treatment,which acts by blocking platelet-derived growth factor receptor-beta kinase activity.Apart from this canonical fusion,there is an expanding spectrum of rare fusions,including COL6A3::PDGFD,EMILIN::PDGFD,TNC::PDGFD,etc.,through mole-cular profiling.These atypical rearrangements may be encountered in morpho-logically classic DFSP,unusual anatomic sites,or diagnostically challenging variants such as fibrosarcomatous DFSP.Their recognition is clinically relevant,as they may influence tumor biology,response to targeted therapy,and eligibility for clinical trials.This newly documented DFSP involving the lacrimal sac was initially misdiagnosed as a solitary fibrous tumor,emphasizing the diagnostic pitfalls in anatomically constrained regions and the importance of integrated diagnosis combining histology,immunohistochemistry,and molecular testing.In this editorial commentary,we briefly highlight the ever-growing genomic land-scape of DFSP,report rare fusions and their biological implications,and examine the role of expanded molecular diagnostics in refining diagnosis,guiding therapy,and informing prognosis.Incorporating comprehensive fusion analysis into routine workup may be critical for accurate classification,especially in unusual presentations where reliance on morphology alone risks misdiagnosis. 展开更多
关键词 dermatofibrosarcoma protuberans COL1A1::PDGFB EMILIN TNC Molecular diagnostics
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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:7
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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 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 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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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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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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以胸椎侵犯为首发表现的纤维肉瘤型隆突性皮肤纤维肉瘤1例
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作者 黎知青 何惠华 吴瑜 《微循环学杂志》 2025年第4期98-101,共4页
目的:探讨胸椎纤维肉瘤型隆突性皮肤纤维肉瘤临床、病理特征和诊断情况。方法:回顾性分析2025年2月本院收治的1例纤维肉瘤型隆突性皮肤纤维肉瘤患者临床资料,对其组织学形态、免疫表型和分子检测结果进行分析,并结合相关文献进行复习总... 目的:探讨胸椎纤维肉瘤型隆突性皮肤纤维肉瘤临床、病理特征和诊断情况。方法:回顾性分析2025年2月本院收治的1例纤维肉瘤型隆突性皮肤纤维肉瘤患者临床资料,对其组织学形态、免疫表型和分子检测结果进行分析,并结合相关文献进行复习总结。结果:患者男,55岁,因“行走不利伴腰部疼痛7月”入院。查体发现左侧腹部、左手大鱼际处可扪及多处大小不等包块。外院曾对胸椎占位进行活检,病理诊断考虑恶性外周神经鞘膜瘤。本院MRI结果显示:T10-12平面椎管内外(偏右侧)占位,结合病史考虑神经源性肿瘤。患者在本院行胸椎占位切除术后病检,镜下见肿瘤细胞呈梭形、形态单一,弥漫片状排列或呈束状及鱼骨状排列,细胞异型性明显,细胞核深染、核分裂易见。结合免疫组化和COL1A1/PDGFB融合基因FISH检测阳性结果,最终诊断为(胸椎肿物)纤维肉瘤型隆突性皮肤纤维肉瘤。结论:胸椎纤维肉瘤型隆突性皮肤纤维肉瘤发生部位罕见,易发生误诊,提高对该病的认识及警惕性有助于实现早期确诊并降低误诊率。 展开更多
关键词 隆突性皮肤纤维肉瘤 病理诊断 肿瘤 胸椎
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CD34和S-100弥漫共表达的隆突性皮肤纤维肉瘤1例
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作者 姚辉 曾英 +3 位作者 姜歆 贾浩 王雪 唐雪峰 《临床与实验病理学杂志》 北大核心 2025年第8期1109-1111,共3页
CD34和S-100弥漫共表达的隆突性皮肤纤维肉瘤罕见,免疫组化易与其他共表达CD34和S-100的肿瘤混淆,应避免相关诊断陷阱。本文报道1例CD34和S-100弥漫共表达的隆突性皮肤纤维肉瘤,镜下见弥漫生长的短梭型和卵圆形细胞,呈束状、鱼骨状排列... CD34和S-100弥漫共表达的隆突性皮肤纤维肉瘤罕见,免疫组化易与其他共表达CD34和S-100的肿瘤混淆,应避免相关诊断陷阱。本文报道1例CD34和S-100弥漫共表达的隆突性皮肤纤维肉瘤,镜下见弥漫生长的短梭型和卵圆形细胞,呈束状、鱼骨状排列;肿瘤细胞分布不均,周边区域可见扩张的薄壁血管;瘤细胞胞质丰富,可见小核仁。免疫组化肿瘤细胞弥漫表达CD34和S-100;二代测序显示COL1A1∷PDGFB融合基因变异。 展开更多
关键词 隆突性皮肤纤维肉瘤 纤维肉瘤亚型 CD34 S-100 病例报道
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儿童隆突性皮肤纤维肉瘤一例
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作者 余平子 李伟泽 +1 位作者 万苗坚 曾义燕 《中国麻风皮肤病杂志》 2025年第6期439-441,共3页
儿童隆突性皮肤纤维肉瘤罕见,易漏诊、误诊。本文报道一例出生时即出现皮损的病例。患儿,男,2岁。左臀部蚕豆大小淡紫色斑块,逐渐增大、增多2年。皮损组织病理示:肿瘤位于真皮及皮下,由梭形或短梭形细胞构成,弥漫或席纹状排列,细胞有异... 儿童隆突性皮肤纤维肉瘤罕见,易漏诊、误诊。本文报道一例出生时即出现皮损的病例。患儿,男,2岁。左臀部蚕豆大小淡紫色斑块,逐渐增大、增多2年。皮损组织病理示:肿瘤位于真皮及皮下,由梭形或短梭形细胞构成,弥漫或席纹状排列,细胞有异型性,可见核分裂。免疫组化示:CD34(+),CD68、CD31、CK、S100、EmA均阴性,Ki-67(约2%+)。诊断:隆突性皮肤纤维肉瘤。治疗:MMS显微描记手术,术后随访无复发。 展开更多
关键词 皮肤纤维肉瘤 隆突性 儿童
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慢Mohs显微描记术联合背阔肌肌皮瓣治疗躯干部巨大隆突性皮肤纤维肉瘤二例并文献复习
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作者 谢凯 王红蕾 +4 位作者 谢卫星 王学庆 曹楠 张广亮 武宜雷 《中国麻风皮肤病杂志》 2025年第2期123-126,共4页
目的:明确慢Mohs显微描记术联合背阔肌肌皮瓣治疗上躯干部巨大隆突性皮肤纤维肉瘤的疗效。方法:对采用慢Mohs显微描记术联合背阔肌肌皮瓣治疗的隆突性皮肤纤维肉瘤2例患者进行回顾性分析。结果:2例患者经一期慢Mohs手术后均获得阴性病... 目的:明确慢Mohs显微描记术联合背阔肌肌皮瓣治疗上躯干部巨大隆突性皮肤纤维肉瘤的疗效。方法:对采用慢Mohs显微描记术联合背阔肌肌皮瓣治疗的隆突性皮肤纤维肉瘤2例患者进行回顾性分析。结果:2例患者经一期慢Mohs手术后均获得阴性病理切缘,术后创面分别为10 cm×13 cm、15 cm×15 cm。均给予二期设计背阔肌肌皮瓣修复原发缺损,继发缺损直接闭合修复。患者皮瓣均全部成活,创面一期愈合。结论:慢Mohs显微描记术是治疗隆突性皮肤纤维肉瘤一线选择,肿瘤切除后躯干部大面积创面可采用背阔肌肌皮瓣修复创面。 展开更多
关键词 隆突性皮肤纤维肉瘤 Mohs显微描记术 背阔肌肌皮瓣
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隆突性皮肤纤维肉瘤72例临床病理学观察 被引量:33
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作者 任晓冰 王坚 +3 位作者 沈磊 俞友娟 陈维 朱雄增 《临床与实验病理学杂志》 CAS CSCD 2003年第1期35-39,共5页
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... 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。 展开更多
关键词 隆突性皮肤纤维肉瘤 DFSP 临床病理学 诊断 CD34 皮肤肿瘤
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超声诊断隆突性皮肤纤维肉瘤 被引量:22
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作者 敬基刚 彭玉兰 +1 位作者 罗燕 李永忠 《中国医学影像技术》 CSCD 北大核心 2009年第10期1830-1832,共3页
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... 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。 展开更多
关键词 隆突性皮肤纤维肉瘤 超声检查 病理
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36例隆突性皮肤纤维肉瘤临床诊疗分析 被引量:15
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作者 练慧斌 姚刚 +3 位作者 周芳 马来祥 陈默轩 章宏伟 《南京医科大学学报(自然科学版)》 CAS CSCD 北大核心 2009年第8期1181-1184,共4页
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... 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。 展开更多
关键词 隆突性皮肤纤维肉瘤 扩大切除 近距离放射治疗
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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 被引量:6
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作者 郭立新 杨光华 +2 位作者 姜勇 李俸媛 廖殿英 《临床与实验病理学杂志》 CAS CSCD 2004年第2期131-135,共5页
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% ... 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。 展开更多
关键词 肌样型隆突性皮肤纤维肉瘤 肌样区域 组织发生 免疫表型 诊断
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