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 720L2

2024/08 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。

• 2026-08病例报告
  Lesions Reported as Plaque-Like CD34-Positive Dermal Fibroma Reveal Two Reproducible Clinicopathologic Patterns: Two Cases and Literature Review
  Journal of cutaneous pathology · DOI · Europe PMC
• 2026-08病例报告
  PDGFD-Rearranged Dermatofibrosarcoma Protuberans With S100 and Pan-TRK Expression
  Journal of cutaneous pathology · DOI · Europe PMC
• 2026-07
  A High-Grade CD34-Positive Spindle Cell Sarcoma of the Palm Revealing an Unexpected TPM3::NTRK1 Fusion: A Diagnostic Pitfall With Major Therapeutic Implications
  Applied immunohistochemistry & molecular morphology : AIMM · DOI · Europe PMC
• 2026-07病例报告
  Dermatofibrosarcoma protuberans following penetrating trauma: Case report
  Radiology case reports · DOI · Europe PMC
• 2026-07
  Diagnostic Pitfalls of Morphological Mimicry and CD34 Heterogeneity in Recurrent or Fibrosarcomatous Dermatofibrosarcoma Protuberans: A Clinicopathological Analysis of Three Molecularly Confirmed Cases With Literature Review
  Journal of cutaneous pathology · DOI · Europe PMC
• 2026-07
  Tumor Characteristics, Subclinical Extent, and Time to Poor Outcomes for less Common Skin Cancers
  Dermatologic surgery : official publication for American Society for D · DOI · Europe PMC
• 2026-07
  Recurrent TNC::PDGFD Fusions in Ossifying Plexiform Tumors of the Skin
  Modern pathology : an official journal of the United States and Canadi · DOI · Europe PMC
• 2026-07
  Mechanism of MEK1 phosphorylation by the N-terminal acidic motif-mediated asymmetric BRAF dimer
  Molecular cell · DOI · Europe PMC
• 2026-07病例报告
  Fibrosarcomatous Transformation of Recurrent Thigh Dermatofibrosarcoma Protuberans Presenting With Spontaneous Hemorrhage
  Plastic and reconstructive surgery. Global open · DOI · Europe PMC
• 2026-07
  Impact of Dermatology Access on Dermatofibrosarcoma Protuberans Outcomes
  Dermatologic surgery : official publication for American Society for D · DOI · Europe PMC
• 2026-07
  MRI-Based Surgical Margin Assessment in Dermatofibrosarcoma Protuberans: Correlation With Pathological Extent
  The Journal of dermatology · DOI · Europe PMC
• 2026-07综述
  Mechanisms of Local Aggressiveness Without Metastasis in Dermatofibrosarcoma Protuberans
  Anticancer research · DOI · Europe PMC
• 2026-07
  Neoadjuvant BO-112 and Hypofractionated Radiation Therapy with or without Nivolumab in Soft-Tissue Sarcoma: Preclinical and Phase I Results
  Cancer discovery · DOI · Europe PMC
• 2026-06
  Proteomics-based approach reveals the involvement of spliceosomal components SF3B and SerpinB9 in dermatofibrosarcoma protuberans
  Orphanet journal of rare diseases · DOI · Europe PMC
• 2026-06
  Congenital Atrophic Dermatofibrosarcoma Protuberans: A Case Report and Narrative Review of the Literature
  Clinical case reports · DOI · Europe PMC
• 2026-06病例报告
  The 'CIED Team' Approach to Device-Related Complications
  JACC. Case reports · DOI · Europe PMC
• 2026-06
  Colonic metastasis of dermatofibrosarcoma protuberans detected by colonoscopy
  Clinical journal of gastroenterology · DOI · Europe PMC
• 2026-06开放获取
  Sorafenib Restores Pentose Phosphate Pathway-Related Redox Homeostasis via the c-Raf/HSP90/G6PD Axis in Hepatic Ischemia-Reperfusion Injury
  MedComm · DOI · Europe PMC
• 2026-06病例报告
  Spindle cell hemangioma of the lower abdominal wall mimicking dermatofibrosarcoma protuberans: A case report
  JAAD case reports · DOI · Europe PMC
• 2026-06病例报告
  Durable control of recurrent dermatofibrosarcoma protuberans using wide-margin Mohs micrographic surgery and flap reconstruction without adjuvant radiotherapy: a case report and literature review
  International journal of surgery case reports · 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）的药品与孤儿药资格公开导出表，以及美国 FDA 孤儿药资格数据库。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