隆凸性皮肤纤维肉瘤

定义 英文原文（暂无中文）

Dermatofibrosarcoma protuberans (DFSP) is a rare infiltrating soft tissue sarcoma, generally of low grade malignancy, arising from the dermis of the skin and characteristically associated with a specific chromosomal translocation t(17;22).

别名

DFSP

基本事实

遗传方式

不适用

发病年龄

各年龄段

患病率

1-5 / 10 000（Europe）

相关基因 2

临床表型 8

近两年的全球研究 743L2

2024/10 起在 Europe PMC 检索所得，按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务，尚未引入。

• 2026-09开放获取
  Three Progressive Upper Trunk Nodules: A Quiz
  Acta dermato-venereologica · DOI · Europe PMC
• 2026-09开放获取
  Accelerated Pancreatic Volume Loss as a Potential Pre-Diagnostic Imaging Biomarker for Pancreatic Cancer Risk Enrichment: An AI-Assisted Volumetric Study
  Cancers · DOI · Europe PMC
• 2026-09病例报告
  Storiform Collagenomas With Unusual Morphology: Expanding the Histologic Spectrum in Two Molecularly Proven Cases
  Journal of cutaneous pathology · DOI · Europe PMC
• 2026-09综述开放获取
  Contemporary Multimodal Management of Cutaneous Malignancies: Surgery, Radiotherapy, Systemic Therapy, Molecular Targeting and Immunotherapy
  Cancers · DOI · Europe PMC
• 2026-09综述开放获取
  Next-Generation Sequencing in Translational Sarcoma Research: Biological Insights and Emerging Directions
  Current oncology reports · DOI · Europe PMC
• 2026-09
  Autoclosing Excision Design for Dermatofibrosarcoma Protuberans in Slow Mohs Surgery
  Clinical case reports · DOI · Europe PMC
• 2026-09病例报告开放获取
  Spindle Cell Tumors with a <i>GAB1::ABL1</i> Fusion-Another Member of Protein-Kinase-Related Soft Tissue Neoplasms: An (Epi)Genetic Study of Six Cases with Benign Behavior
  International journal of molecular sciences · DOI · Europe PMC
• 2026-09
  A deceptive blue nodule: pigmented dermatofibrosarcoma protuberans
  QJM : monthly journal of the Association of Physicians · DOI · Europe PMC
• 2026-09综述开放获取
  Basic Principles of Skin Biopsy Optimization in Dermatopathology
  Dermatopathology (Basel, Switzerland) · DOI · Europe PMC
• 2026-09
  People of Color With Dermatofibrosarcoma Protuberans Experience High Rates of Fibrosarcomatous Change but No Local Recurrences After Mohs Micrographic Surgery
  International journal of dermatology · DOI · Europe PMC
• 2026-09开放获取
  Autoclosing Excision Design for Dermatofibrosarcoma Protuberans in Slow Mohs Surgery
  Clinical case reports
• 2026-09开放获取
  Rekonstruktion großer Defekte an Kopfhaut und Stirn mittels Transpositionslappen und Gitarrensaitennähten. Eine monozentrische retrospektive Studie
  Journal der Deutschen Dermatologischen Gesellschaft = Journal of the G
• 2026-09病例报告开放获取
  Intraoral desmoplastic melanoma requiring radical multidisciplinary resection and free flap reconstruction: case report and literature review
  Journal of surgical case reports · DOI · Europe PMC
• 2026-09综述开放获取
  An Update on Selected Giant Cell-Rich Tumors of Soft Tissue
  Cancer genomics & proteomics · DOI · Europe PMC
• 2026-08开放获取
  Use of receptor and nonreceptor tyrosine kinase inhibitors and Parkinson disease risk
  Neurotherapeutics : the journal of the American Society for Experiment · DOI · Europe PMC
• 2026-08综述开放获取
  Pediatric sarcomas: challenges and opportunities
  Genes & development · DOI · Europe PMC
• 2026-08综述
  Features of Skin of Color
  Deutsches Arzteblatt international · DOI · Europe PMC
• 2026-08病例报告开放获取
  Giant Pilomatrixoma: Report of Two Cases and Review of Diagnostic and Therapeutic Approach
  Cureus · DOI · Europe PMC
• 2026-08
  Clinicopathological analysis of distant metastasis in dermatofibrosarcoma protuberans: a report of 14 cases
  Journal der Deutschen Dermatologischen Gesellschaft = Journal of the G · DOI · Europe PMC
• 2026-08开放获取
  Is the CALLY Index the Best Tool for Predicting Survival in Patients with Primary Soft Tissue Sarcomas?
  Diagnostics (Basel, Switzerland) · DOI · Europe PMC

境外已获批用于本病的药物 1L2

欧盟 0 项、美国 1 项。同一药物在两地各批一次的，会分别列出。

• Gleevec美国2006-10-19
  imatinib mesylate
  官方记录

数据来自欧洲药品管理局（EMA）的药品与罕见病用药资格公开导出表，以及美国 FDA 的罕见病用药资格数据库（Orphan Drug Product Designation Database）。两边口径不同：欧盟一侧取的是当前状态仍为「已授权」的药品；美国一侧记录的是「曾获批准」这一事实，FDA 的公开表不追踪药物此后是否退市（例如 Relyvrio 于 2024 年撤市，表中仍记为已获批）。请以官方记录页为准。

中国境外的在招试验 9L2

共 9 项。

• 尚未开始招募NCT07646080
  Exploratory Study on Toripalimab and Anlotinib Combined With Standard Chemotherapy for Refractory Dermatofibrosarcoma Protuberans
  II 期 · 干预性 · 2026/06/15Yanjie Zhang, MD
• 招募中NCT07173972
  Dose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma.
  II 期 · 干预性 · 2026/01/26Oslo University Hospital
  挪威
• 招募中NCT07169344
  Hypofractionated, 3-week, Preoperative Proton or X-ray Radiotherapy for Patients With Localized Soft Tissue Sarcoma
  II 期 · 干预性 · 2026/01/26Oslo University Hospital
  挪威
• 尚未开始招募NCT06526897
  Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma
  不适用 · 干预性 · 2025/01/28ECOG-ACRIN Cancer Research Group
• 招募中NCT06789172
  A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors
  I 期 · 干预性 · 2025/01/23Epkin
  澳大利亚、英国、美国
• 尚未开始招募NCT06610071
  NIFR Image-guided Surgery for Malignant Soft Tissue Tumor With Low-dose SWIG Technique
  I 期 · 干预性 · 2024/10/30The Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College
• 招募中NCT06239272
  NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
  I 期、II 期 · 干预性 · 2024/03/27St. Jude Children's Research Hospital
  美国
• 招募中NCT05711615
  Testing Low-Dose Common Chemotherapy (Liposomal Doxorubicin) in Combination With an Anti-Cancer Drug, Peposertib, in Advanced Sarcoma
  I 期 · 干预性 · 2024/02/06National Cancer Institute (NCI)
  美国
• 招募中NCT04332874
  A Study of Pembrolizumab Plus Local Chemotherapy Using Isolated Limb Infusion (ILI) for Patients With Sarcoma in the Arm or Leg
  II 期 · 干预性 · 2020/04/01Memorial Sloan Kettering Cancer Center
  美国

外部标识与链接