ArticleOrphanet journal of rare diseases2017
Identification of EML4-ALK as an alternative fusion gene in epithelioid inflammatory myofibroblastic sarcoma.
Article in Orphanet journal of rare diseases, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 40 citations in OpenAlex.
- Inflammatory myofibroblastic tumour: unravelling the interplay of inflammation, kinase signalling, and therapeutic resistance.Frontiers in oncology · 2026Review
- Thoracic epithelioid inflammatory myofibroblastic sarcoma: a rare and aggressive disease with case report and literature review.Discover oncology · 2024Article
- Primary epithelioid inflammatory myofibroblastic sarcoma of the brain with EML4::ALK fusion mimicking intra-axial glioma: a case report and brief literature review.Journal of pathology and translational medicine · 2024Article
- Epithelioid inflammatory myofibroblastic sarcoma treated with Alectinib: a case report and literature review.Frontiers in oncology · 2024Article
- Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment.Oncology research · 2024Review
- Mediastinal epithelioid inflammatory myofibroblastic sarcoma with theRespirology case reports · 2024Article
- A case report: Pathological complete response to neoadjuvant lorlatinib for Epithelioid inflammatory myofibroblastic sarcoma with EML4-ALK rearrangement.Frontiers in pharmacology · 2024Article
- Rare giant epithelioid inflammatory myofibroblastic sarcoma of the abdominal cavity in a child: a case report and review of the literature.Frontiers in oncology · 2024Article
- Epithelioid inflammatory myofibroblastic sarcoma with exceptionally long response to lorlatinib-a case report.Therapeutic advances in medical oncology · 2024Article
- Article
- Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib.Frontiers in oncology · 2023Article
- Epithelioid inflammatory myofibroblastic sarcoma: a case report and brief literature review.Frontiers in oncology · 2023Article
- Inflammatory Myofibroblastic Tumour: State of the Art.Cancers · 2022Review
- Systems-Level Mapping of Cancer Testis Antigen 1b/a to Sarcoma Pathways Identifies Activated Ran Binding-2 E3 SUMO-Protein Ligase and Transducin-Like Enhancer Protein 1.Frontiers in genetics · 2022Article
- Epithelioid inflammatory myofibroblastic sarcoma with VCL-ALK fusion of central nervous system: case report and brief review of the literature.Brain tumor pathology · 2022Review
- Case Report: Early Distant Metastatic Inflammatory Myofibroblastic Tumor HarboringFrontiers in medicine · 2022Article
- Durable Clinical Response to ALK Tyrosine Kinase Inhibitors in Epithelioid Inflammatory Myofibroblastic Sarcoma HarboringFrontiers in oncology · 2022Article
- Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review.JPGN reports · 2021Review
- Crizotinib in Sarcomatous Malignancies Harboring ALK Fusion With a Definitive Partner(s): Response and Efficacy.Frontiers in oncology · 2021Review
- Crizotinib and Surgery for Long-Term Disease Control in Children and Adolescents With ALK-Positive Inflammatory Myofibroblastic Tumors.JCO precision oncology · 2019Article
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Authors and funding
9 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundKnown as solid tumors of intermediate malignant potential, most inflammatory myofibroblastic tumors (IMTs) are treatable as long as the tumor is en-bloc resected. However, in some cases, the tumors have recurred and grown rapidly after successful surgery. Some of these tumors were classified as an epithelioid inflammatory myofibroblastic sarcoma (EIMS). Most previously reported EIMSs have been caused by RANBP2-ALK fusion gene. We herein report an EIMS case caused by an EML4-ALK fusion gene.
methodsRNAseq was conducted to find out the new ALK fusion gene which could not be detected following previously reported RT-PCR methods for EIMS cases with RANBP2-ALK fusion gene. After that, RT-PCR was also conducted to further prove the newly found fusion gene. Immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) test were applied to find out the unique morphological characters compared with the previous reported EIMS cases.
resultsWe found an EIMS case who was suffering from a rapid recurrence after cytoreducyive surgery was done to relieve the exacerbating symptoms. The patient finally died for tumor lysis syndrome after the application of crizotinib. Distinctive ALK staining under the membrane and relatively weak ALK staining in the cytoplasm could also be observed. RNAseq and RT-PCR further revealed that the tumor harbored an EML4-ALK fusion gene.
conclusionIn conclusion, this is the first EIMS demonstrated to have been caused by the formation of an EML4-ALK fusion gene. This enriches the spectrum of EIMS and enlarges the horizon for the study of EIMS. The experience we shared in managing this kind of disease by discussing aspects of its success and failure could be of great value for surgeons and pathologists.
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