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炎性肌纤维母细胞瘤

Inflammatory myofibroblastic tumor

ORPHA:178342疾病中国目录 第2批 · 42

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

基因名称关联类型
TPM3tropomyosin 3Part of a fusion gene in
ALKALK receptor tyrosine kinasePart of a fusion gene in
RANBP2RAN binding protein 2Part of a fusion gene in
CLTCclathrin heavy chainPart of a fusion gene in
CARS1cysteinyl-tRNA synthetase 1Part of a fusion gene in
TPM4tropomyosin 4Part of a fusion gene in

近两年的全球研究 711L2

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
    Detection of an inflammatory myofibroblastic tumor in the testis by FDG-PET/CT; a rare presentation of a rare neoplasm
    Seminars in nuclear medicine · DOI · Europe PMC
  • 2026-08
    Robot-assisted surgery for combined mini-invasive hemivertebrectomy with thoracotomy for en bloc resection of a paravertebral primary thoraco-lumbar tumor: technical note
    Neuro-Chirurgie · DOI · Europe PMC
  • 2026-08
    Uterine Tumor With Myogenic Differentiation and SRF::RELA Fusion Manifesting as Endometrial Polyp
    International journal of gynecological pathology : official journal of · DOI · Europe PMC
  • 2026-08病例报告
    [Invasive uterine inflammatory myofibroblastic tumor with TNS1::ALK fusion: report of a case]
    Zhonghua bing li xue za zhi = Chinese journal of pathology · DOI · Europe PMC
  • 2026-07
    Expanding the Spectrum of Cutaneous ALK Fusion Mesenchymal Tumors: A Case with Unique Histological Features
    The American Journal of dermatopathology · DOI · Europe PMC
  • 2026-07病例报告
    Surgical and Conservative Management of Periorbital Tumors in Four Dogs
    Veterinary sciences · DOI · Europe PMC
  • 2026-07
    Clinicopathological Features and Expression of CD30, OCT2, and Cyclin D1 in Rosai-Dorfman Disease: A Retrospective Case Series
    Applied immunohistochemistry & molecular morphology : AIMM · DOI · Europe PMC
  • 2026-07
    Rapidly enlarging intraocular inflammatory myofibroblastic tumor in an adult
    Immunological medicine · DOI · Europe PMC
  • 2026-07病例报告
    First Reported Pediatric Bladder Inflammatory Myofibroblastic Tumor (IMT) Evaluated and Staged with VI-RADS: CT and MR Findings
    Reviews on recent clinical trials · DOI · Europe PMC
  • 2026-07
    Clinical impact of second opinion consultation in bladder biopsies and transurethral resection of bladder tumors
    Human pathology · DOI · Europe PMC
  • 2026-07病例报告
    Gastrointestinal Mucormycosis Masquerading as an Abdominal Mass in an Immunocompetent Child
    Journal of Indian Association of Pediatric Surgeons · DOI · Europe PMC
  • 2026-07
    18F-FDG PET/CT Imaging of Multiple Inflammatory Myofibroblastic Tumors
    Clinical nuclear medicine · DOI · Europe PMC
  • 2026-06病例报告
    Management of Inflammatory Myofibroblastic Tumor of Urinary Bladder With Partial Cystectomy: A Case Report
    Cureus · DOI · Europe PMC
  • 2026-06病例报告
    A Case Report of Anaplastic Lymphoma Kinase-Negative Inflammatory Myofibroblastic Tumor in the Anterior Mediastinum of an Elderly Male
    Journal of visualized experiments : JoVE · DOI · Europe PMC
  • 2026-06开放获取
    IgG4-related inflammatory pseudotumor presenting with refractory seizures: a diagnostic pitfall
    Arquivos de neuro-psiquiatria
  • 2026-06开放获取
    Frequency, Clinical Characteristics, Anatomical Distribution, and Outcomes of Embolic Complications of Cardiac Myxoma in Contemporary Cohorts: Protocol for a Systematic Review and Meta-Analysis
    JMIR research protocols · DOI · Europe PMC
  • 2026-06综述病例报告
    Mandibular Inflammatory Myofibroblastic Tumors: A Literature Review with a New Case Presentation
    Current oncology (Toronto, Ont.) · DOI · Europe PMC
  • 2026-06病例报告
    An unusual presentation of abdominopelvic inflammatory myofibroblastic tumor in an adolescent male: a rare case report
    International journal of surgery case reports · DOI · Europe PMC
  • 2026-06病例报告开放获取
    Endobronchial Epstein-Barr Virus-Associated Smooth Muscle Tumor in Advanced HIV Infection: A Rare Case
    The American journal of case reports · DOI · Europe PMC
  • 2026-06病例报告开放获取
    AngioVac aspiration as a combined diagnostic and therapeutic strategy for an indeterminate right atrial mass in a patient with malignancy
    Radiology 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。

「境外已获批」不等于「在中国能用」。中间隔着进口注册、临床急需境外新药通道、海南博鳌乐城国际医疗旅游先行区等几条路径,各有各的条件与费用。这一节能确定地告诉你的只有一件事:这个病在世界范围内已经有获得批准的药物,它叫什么名字。拿这个名字去问主治医生,是下一步最省力的做法。

药名一律保留英文原文,不作翻译——中国的药品通用名与英文名的音译经常不一致,译错会让人去找一个不存在的药。

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。

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。

其他状态的试验(3 项)
  • 已完成NCT02971176
    Low-dose Protocol for Computed Tomography-guided Lung Biopsy
    不适用 · 干预性 · 2016/11/30Xuzhou Central Hospital
    中国研究中心 1 个:Xuzhou
  • 状态未知NCT04572594
    Molecular Profiling and Molecular Labeling of Inflammatory Myofibroblastic Tumor
    观察性 · 2020/01/01Sun Yat-sen University
    中国研究中心 1 个:Guangzhou
  • 进行中·不再招募NCT05160922
    Crizotinib Continuation Clinical Study
    IV 期 · 干预性 · 2021/12/27Pfizer
    中国研究中心 9 个:Beijing、Changchun、Fuzhou、Guangzhou、Hangzhou、Shanghai 等 7 地

中国境外的在招试验 3L2

这些试验在中国没有研究中心,通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处:看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生,或据此进一步查该药是否已在境外获批、是否有拓展性用药(expanded access)通道。

美国2法国2荷兰1澳大利亚1加拿大1丹麦1意大利1新加坡1韩国1西班牙1中国台湾1英国1

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

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本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)