ArticleThe Journal of pathology2019
NR4A3 fusion proteins trigger an axon guidance switch that marks the difference between EWSR1 and TAF15 translocated extraskeletal myxoid chondrosarcomas.
Article in The Journal of pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- Epithelioid and spindle rhabdomyosarcoma with TFCP2 rearrangement in abdominal wall: a distinctive entity with poor prognosis.Diagnostic pathology · 2023Pooled it
- Clinical and magnetic resonance imaging features of soft tissue extraskeletal myxoid chondrosarcoma: A retrospective observational cohort study.Skeletal radiology · 2026Observational
- Extraskeletal Myxoid Chondrosarcoma Mimicking Myoepithelial Tumor.Case reports in pathology · 2026Article
- Chondrosarcoma: New Molecular Insights, Challenges in Near-Patient Preclinical Modeling, and Therapeutic Approaches.International journal of molecular sciences · 2025Review
- Potential Roles of Nr4a3-Mediated Inflammation in Immunological and Neurological Diseases.Molecular neurobiology · 2024Review
- Identification of Novel/RareInternational journal of molecular sciences · 2024Article
- Extraskeletal myxoid chondrosarcoma of the gingival: a rare case report and review of the literature.Diagnostic pathology · 2023Review
- HOXA5 is a key regulator of class 3 semaphorins expression in the synovium of rheumatoid arthritis patients.Rheumatology (Oxford, England) · 2023Article
- Whole genome sequencing for metastatic mutational burden in extraskeletal myxoid chondrosarcoma.Frontiers in molecular medicine · 2023Article
- Family cancer history and smoking habit associated with sarcoma in a Japanese population study.Scientific reports · 2022Article
- Primary intracranial extraskeletal myxoid chondrosarcoma: A case report and review of literature.World journal of clinical cases · 2022Article
- Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma.Scientific reports · 2022Article
- Case Report: Gene Heterogeneity in the Recurrent and Metastatic Lesions of a Myxoid Chondrosarcoma Patient With Aggressive Transformation.Frontiers in genetics · 2022Article
- Extraskeletal Myxoid Chondrosarcoma with Molecularly Confirmed Diagnosis: A Multicenter Retrospective Study Within the Italian Sarcoma Group.Annals of surgical oncology · 2021Article
- A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues.Autopsy & case reports · 2021Article
- Sarcoma treatment in the era of molecular medicine.EMBO molecular medicine · 2020Review
- Extraskeletal Myxoid Chondrosarcoma: State of the Art and Current Research on Biology and Clinical Management.Cancers · 2020Review
- Primary mediastinal chondrosarcomas: do they really exist?Mediastinum (Hong Kong, China) · 2020Review
- Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions.Frontiers in oncology · 2020Article
Corrections and comments
- Erratum issued
Authors and funding
21 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Extraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma histotype with uncertain differentiation. EMC is hallmarked by the rearrangement of the NR4A3 gene, which in most cases fuses with EWSR1 or TAF15. TAF15-translocated EMC seem to feature a more aggressive course compared to EWSR1-positive EMCs, but whether the type of NR4A3 chimera impinges upon EMC biology is still largely undefined. To gain insights on this issue, a series of EMC samples (7 EWSR1-NR4A3 and 5 TAF15-NR4A3) were transcriptionally profiled. Our study unveiled that the two EMC variants display a distinct transcriptional profile and that the axon guidance pathway is a major discriminant. In particular, class 4-6 semaphorins and axonal guidance cues endowed with pro-tumorigenic activity were more expressed in TAF15-NR4A3 tumors; vice versa, class 3 semaphorins, considered to convey growth inhibitory signals, were more abundant in EWSR1-NR4A3 EMC. Intriguingly, the dichotomy in axon guidance signaling observed in the two tumor variants was recapitulated in in vitro cell models engineered to ectopically express EWSR1-NR4A3 or TAF15-NR4A3. Moreover, TAF15-NR4A3 cells displayed a more pronounced tumorigenic potential, as assessed by anchorage-independent growth. Overall, our results indicate that the type of NR4A3 chimera dictates an axon guidance switch and impacts on tumor cell biology. These findings may provide a framework for interpretation of the different clinical-pathological features of the two EMC variants and lay down the bases for the development of novel patient stratification criteria and therapeutic approaches. © 2019 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.
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