炎性肌纤维母细胞瘤

定义

炎性肌纤维母细胞瘤是一种罕见的黏膜下间质瘤，由梭形细胞、肌成纤维细胞和炎性细胞组成，呈异质性，一般为良性，但也可发生局部侵袭、复发、恶性转化或血管侵犯和转移，具体与累及的器官有关。该病可发生于任何器官，常发生于肺、肠系膜、网膜和腹膜后，部分患者可出现副肿瘤综合征（发热、不适、体重减轻、贫血、血小板增多）或邻近器官受压症状，如肠梗阻。

相关基因 6

近两年的全球研究 759L2

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综述病例报告开放获取
  Primary ALK-positive anaplastic large cell lymphoma of the urinary bladder in a pediatric patient: a case report and review of literature
  Journal of hematopathology · DOI · Europe PMC
• 2026-09开放获取
  Noninvasive Preoperative Stratification of Renal Tumors for Aggressiveness and Prognosis
  Bioengineering (Basel, Switzerland) · 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开放获取
  Intra-Abdominal Myxoid Leiomyosarcoma With PLAG1 Gene Rearrangement Following Prior Laparoscopic Myomectomy: A Case Report and Literature Review
  Clinical case reports · DOI · Europe PMC
• 2026-09病例报告开放获取
  Sclerosing angiomatoid nodular transformation of the spleen in an 18-year-old man: a case report
  Journal of surgical case reports · DOI · Europe PMC
• 2026-09
  Pulmonary Inflammatory Myofibroblastic Tumor Mimicking Septic Pulmonary Embolism Following a Dental Infection
  Internal medicine (Tokyo, Japan) · DOI · Europe PMC
• 2026-09开放获取
  An individualized reconstructive algorithm for large facial defects after tumor resection in elderly patients: A retrospective case series
  JPRAS open · DOI · Europe PMC
• 2026-09综述
  Mesenchymal Neoplasms of the Pancreatobiliary Tract
  Archives of pathology & laboratory medicine · DOI · Europe PMC
• 2026-09综述
  Inflammatory Soft Tissue Tumors: A Wolf in Sheep's Clothing
  Archives of pathology & laboratory medicine · DOI · Europe PMC
• 2026-09病例报告开放获取
  Primary Bronchial Hybrid Low-Grade Fibromyxoid Sarcoma/Sclerosing Epithelioid Fibrosarcoma With EWSR1::CREB3L1 Fusion: Diagnostic Insights From a Challenging Case in the Respiratory Tract
  Pathology international · DOI · Europe PMC
• 2026-09
  Nano-RAPID: Integrated Diagnosis for Sarcoma Using a Nanopore Sequencer
  Genes, chromosomes & cancer · DOI · Europe PMC
• 2026-09病例报告
  Acral Fibrochondromyxoid Tumor: Diagnostic Challenges and Multimodal Characterization in a Hand Mass Case
  Pathology international · DOI · Europe PMC
• 2026-09开放获取
  Priming Cells with Modulators of Cellular Signaling Ensures Enhanced Selectivity of HSPG-Specific Protein-Drug Conjugate Delivery
  Journal of the American Chemical Society · DOI · Europe PMC
• 2026-09病例报告
  Pelvic ALK-Positive Inflammatory Myofibroblastic Tumor Presenting 8 Years After Uterine Smooth Muscle Tumor of Uncertain Malignant Potential
  The journal of obstetrics and gynaecology research · DOI · Europe PMC
• 2026-09病例报告
  [Cutaneous inflammatory myofibroblastic tumor with CARS::ALK fusion: report of a case]
  Zhonghua bing li xue za zhi = Chinese journal of pathology · DOI · Europe PMC
• 2026-09病例报告开放获取
  Endobronchial epithelioid leiomyoma (leiomyoblastoma) causing complete right-lung atelectasis: A case report
  Medicine · DOI · Europe PMC
• 2026-09综述病例报告开放获取
  Inflammatory Myofibroblastic Tumor of the Urinary Bladder Presenting as a Radiological cT3 Mass: A Case Report and Narrative Review of 89 Cases
  In vivo (Athens, Greece) · DOI · Europe PMC
• 2026-09开放获取
  Not all NTRK fusions in mesenchymal neoplasia are driver events: implications on classification and targeted therapy
  The journal of pathology. Clinical research · DOI · Europe PMC
• 2026-08开放获取
  What's new in neoplastic pulmonary pathology 2026: updates on grading, staging and molecular testing
  Journal of pathology and translational medicine · DOI · Europe PMC
• 2026-08病例报告开放获取
  Primary sarcomatoid urothelial carcinoma of the ureter: A case report and literature review
  Urology 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。

• Xalkori美国2022-07-14
  crizotinib
  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。

在中国开展的临床试验 3L2

按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实，并与主治医生商量。

当前没有检索到登记为可入组的试验。

中国境外的在招试验 3L2

共 3 项。

• 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NCT04925609
  Brigatinib in Pediatric and Young Adult Patients With ALK+ ALCL, IMT or Other Solid Tumors
  I 期、II 期 · 干预性 · 2022/08/18Princess Maxima Center for Pediatric Oncology
  法国、荷兰
• 招募中NCT04094610
  A Study of Repotrectinib in Pediatric and Young Adult Subjects Harboring ALK, ROS1, OR NTRK1-3 Alterations
  I 期、II 期 · 干预性 · 2020/03/12Turning Point Therapeutics, Inc.
  澳大利亚、加拿大、丹麦、法国、意大利、新加坡、韩国、西班牙 等 11 国

外部标识与链接