Skip to main content
SpringerLink
Log in

Myxoid Chondosarcoma, Extraskeletal

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 21 Accesses

Definition

Extraskeletal myxoid chondrosarcoma is defined as a malignant mesenchymal neoplasm of uncertain differentiation, displaying abundant myxoid matrix, a lobulated architecture and cords and strands of uniform cells, characterized by NR4A3 gene rearrangements. The term “chondrosarcoma” was originally chosen based on the positivity for S100, the resemblance to chondroblasts at electron microscopy, and on focal cartilage formation. The latter is however extremely rare, as is the expression of cartilaginous differentiation markers, and it is now generally accepted that the term “chondrosarcoma” in extraskeletal myxoid chondrosarcoma is a misnomer. Therefore, since the 2002 WHO classification, the tumor is classified in the group of “of uncertain differentiation.” With emerging genetics, “NR4A3- rearranged myxoid sarcoma” might be a more appropriate term.

Clinical Features

  • Incidence

    Extraskeletal myxoid chondrosarcoma is rare and represents <1% of all soft tissue sarcomas.

    ...

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References and Further Reading

  • Agaram, N. P., Zhang, L., Sung, Y. S., Singer, S., & Antonescu, C. R. (2014). Extraskeletal myxoid chondrosarcoma with non-EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology. Human Pathology, 45(5), 1084–1091.

    Article  CAS  Google Scholar 

  • Attwooll, C., Tariq, M., Harris, M., Coyne, J. D., Telford, N., & Varley, J. M. (1999). Identification of a novel fusion gene involving hTAFII68 and CHN from a t(9;17)(q22;q11.2) translocation in an extraskeletal myxoid chondrosarcoma. Oncogene, 18(52), 7599–7601.

    Article  CAS  Google Scholar 

  • Brenca, M., Stacchiotti, S., Fassetta, K., et al. (2019). NR4A3 fusion proteins trigger an axon guidance switch that marks the difference between EWSR1 and TAF15 translocated extraskeletal myxoid chondrosarcomas. The Journal of Pathology, 249(1), 90–101.

    Article  CAS  Google Scholar 

  • Clark, J., Benjamin, H., Gill, S., et al. (1996). Fusion of the EWS gene to CHN, a member of the steroid thyroid receptor gene superfamily, in a human myxoid chondrosarcoma. Oncogene, 12, 229–235.

    CAS  PubMed  Google Scholar 

  • Finos, L., Righi, A., Frisoni, T., et al. (2017). Primary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature. Pathology, Research and Practice, 213(5), 461–466.

    Article  CAS  Google Scholar 

  • Kawaguchi, S., Wada, T., Nagoya, S., et al. (2003). Extraskeletal myxoid chondrosarcoma: A multi-institutional study of 42 cases in Japan. Cancer, 97(5), 1285–1292.

    Article  Google Scholar 

  • Kohashi, K., Oda, Y., Yamamoto, H., et al. (2008). SMARCB1/INI1 protein expression in round cell soft tissue sarcomas associated with chromosomal translocations involving EWS: A special reference to SMARCB1/INI1 negative variant extraskeletal myxoid chondrosarcoma. The American Journal of Surgical Pathology, 32(8), 1168–1174.

    Article  Google Scholar 

  • Meis-Kindblom, J. M., Bergh, P., Gunterberg, B., & Kindblom, L. G. (1999). Extraskeletal myxoid chondrosarcoma: A reappraisal of its morphologic spectrum and prognostic factors based on 117 cases. The American Journal of Surgical Pathology, 23(6), 636–650.

    Article  CAS  Google Scholar 

  • Panagopoulos, I., Mertens, F., Isaksson, M., et al. (2002). Molecular genetic characterization of the EWS/CHN and RBP56/CHN fusion genes in extraskeletal myxoid chondrosarcoma. Genes, Chromosomes & Cancer, 35(4), 340–352.

    Article  CAS  Google Scholar 

  • Patel, S. R., Burgess, M. A., Papadopoulos, N. E., Linke, K. A., & Benjamin, R. S. (1995). Extraskeletal myxoid chondrosarcoma. Long-term experience with chemotherapy. American Journal of Clinical Oncology, 18(2), 161–163.

    Article  CAS  Google Scholar 

  • Sciot, R., Dal Cin, P., Fletcher, C., et al. (1995). T(9;22)(q22-31;q11-12) is a consistent marker of extraskeletal myxoid chondrosarcoma: Evaluation of three cases. Modern Pathology, 8(7), 765–768.

    CAS  PubMed  Google Scholar 

  • Sjogren, H., Meis-Kindblom, J., Kindblom, L. G., Aman, P., & Stenman, G. (1999). Fusion of the EWS-related gene TAF2N to TEC in extraskeletal myxoid chondrosarcoma. Cancer Research, 59(20), 5064–5067.

    CAS  PubMed  Google Scholar 

  • Sjogren, H., Meis-Kindblom, J. M., Orndal, C., et al. (2003). Studies on the molecular pathogenesis of extraskeletal myxoid chondrosarcoma-cytogenetic, molecular genetic, and cDNA microarray analysis. American Journal of Pathology, 162(3), 781–792.

    Article  Google Scholar 

  • Stacchiotti, S., Ferrari, S., Redondo, A., et al. (2019). Pazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: A multicentre, single-arm, phase 2 trial. The Lancet Oncology, 20, 1252–1262.

    Article  CAS  Google Scholar 

  • Stenman, G., Andersson, H., Mandahl, N., Meis-Kindblom, J. M., & Kindblom, L.-G. (1995). Translocation t(9;22)(q22;q12) is a primary cytogenetic abnormality in extraskeletal myxoid chondrosarcoma. International Journal of Cancer, 62, 398–402.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, LUMC, Leiden, The Netherlands

    Judith V. M. G. Bovée

Authors
  1. Judith V. M. G. Bovée
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Judith V. M. G. Bovée .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Bovée, J.V.M.G. (2020). Myxoid Chondosarcoma, Extraskeletal. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5425-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5425-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation