ArticleAnnals of surgical oncology2021
Extraskeletal Myxoid Chondrosarcoma with Molecularly Confirmed Diagnosis: A Multicenter Retrospective Study Within the Italian Sarcoma Group.
Article in Annals of surgical oncology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 32 citations in OpenAlex.
- Clinical and magnetic resonance imaging features of soft tissue extraskeletal myxoid chondrosarcoma: A retrospective observational cohort study.Skeletal radiology · 2026Observational
- Article
- From pathogenesis to the patient's bedside: a comprehensive review of extraskeletal myxoid chondrosarcoma.Journal of cancer research and clinical oncology · 2025Review
- The role of radiotherapy and chemotherapy in extraskeletal myxoid chondrosarcoma.Journal of orthopaedic surgery and research · 2025Article
- Diagnosis and Management of Large Extraosseous Myxoid Chondrosarcoma of the Buttocks: A Case Report.The American journal of case reports · 2025Article
- Establishment and characterization of NCC-EMC1-C1: a novel patient-derived cell line of extraskeletal myxoid chondrosarcoma.Human cell · 2025Article
- Novel liquid biopsy CNV biomarkers in malignant melanoma.Scientific reports · 2024Article
- A nonsteroidal anti-inflammatory drug, zaltoprofen, inhibits the growth of extraskeletal chondrosarcoma cells by inducing PPARγ, p21, p27, and p53.Cell cycle (Georgetown, Tex.) · 2023Article
- Molecular testing of soft tissue tumors.Diagnostic cytopathology · 2023Review
- Metastatic extraskeletal myxoid chondrosarcoma presenting as a forehead mass.JAAD case reports · 2022Article
- Ewing sarcoma and related FET family translocation-associated round cell tumors: A century of clinical and scientific progress.Genes, chromosomes & cancer · 2022Article
- Primary intracranial extraskeletal myxoid chondrosarcoma: A case report and review of literature.World journal of clinical cases · 2022Article
- High-dose-rate interstitial brachytherapy as a suitable option for metastatic extraskeletal myxoid chondrosarcoma - a case report.Journal of contemporary brachytherapy · 2022Article
- Clinical Activity of Single-Agent Cabozantinib (XL184), a Multi-receptor Tyrosine Kinase Inhibitor, in Patients with Refractory Soft-Tissue Sarcomas.Clinical cancer research : an official journal of the American Association for Cancer Research · 2022Article
- Article
- Case Report: Gene Heterogeneity in the Recurrent and Metastatic Lesions of a Myxoid Chondrosarcoma Patient With Aggressive Transformation.Frontiers in genetics · 2022Article
- Biological Heterogeneity of Chondrosarcoma: From (Epi) Genetics through Stemness and Deregulated Signaling to Immunophenotype.Cancers · 2021Review
- Extraskeletal Myxoid Chondrosarcoma: State of the Art and Current Research on Biology and Clinical Management.Cancers · 2020Review
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Authors and funding
16 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundExtraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma of uncertain origin, marked by specific chromosomal translocations involving the NR4A3 gene, and usually characterized by an indolent course. Surgery (with or without radiotherapy) is the treatment of choice in localized disease. The treatment for advanced disease remains uncertain. In order to better evaluate prognostic factors and outcome, a retrospective pooled analysis of patients with EMC treated at three Italian Sarcoma Group (ISG) referral centers was carried out.
methodsAll patients with localized EMC surgically treated from 1989 to 2016 were identified. Diagnosis was centrally reviewed according to WHO 2013. Only patients with NR4A3 rearrangement were included.
resultsSixty-seven patients were identified: 13 (20%) female, 54 (80%) male. Median age was 56 years (range 18-84). Numbers and type of translocation were: 50 (80%) NR4A3-EWS, 10 (16%) NR4A3-TAF15, 1 (2%) NR4A3-TCF12, and 1 (2%) NR4A3-TFG. Median follow-up was 55 months (range 2-312). Five- and ten-year overall survival rates were 94% (86-100 95%CI) and 84% (69-98 95%CI). Thirty-five (52%) patients relapsed: 9 had local recurrence (LR) and 26 had distant metastasis (5 with concomitant LR). The 5- and 10-year disease-free survival rates (DFS) were 51% (38-65 95%CI) and 20% (7-33 95%CI). Size of the primary tumor was significantly related to distant metastasis-free survival (DMFS) (p = 0.004). Patients carrying the NR4A3-EWS translocation had a trend in favor of better DFS (p = 0.08) and DMFS (p = 0.09) compared with the patients with NR4A3-TAF15.
conclusionsProlonged survival can be expected in patients with EMC, in spite of a high rate of recurrence. Size is significantly associated with distant relapse. The type of NR4A3 translocation could influence outcome.
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