Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Mediastinal Extraskeletal Myxoid Chondrosarcoma

  • Annikka WeissferdtEmail author
  • Cesar A. Moran
Reference work entry


Chondrosarcoma of the extraskeletal soft tissues; Chordoid sarcoma; Chordoid tumor; EMC; Extraskeletal chondrosarcoma; Tenosynovial sarcoma


Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue neoplasm characterized by distinct histopathological and molecular features. Although its name implies cartilaginous differentiation, several studies have demonstrated neural or neuroendocrine differentiation in these tumors raising the possibility of polyphenotypic origin. In the latest World Health Organization (WHO) classification of soft tissue and bone tumors, the entity is therefore grouped among the lesions of “uncertain differentiation.” Extraskeletal myxoid chondrosarcoma is primarily a tumor of the deep soft tissues of the lower extremities and limb girdles. Rare tumors have been reported to arise in unusual anatomic locations such as the retroperitoneum, digits, or intracranial cavity. Only a single case has been reported to occur in the posterior...

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References and Further Reading

  1. Enzinger, F. M., & Shiraki, M. (1972). Extraskeletal myxoid chondrosarcoma. An analysis of 34 cases. Human Pathology, 3, 421–435.PubMedCrossRefGoogle Scholar
  2. 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. American Journal of Surgical Pathology, 23, 636–650.PubMedCrossRefGoogle Scholar
  3. Ogura, K., Fujiwara, T., Beppu, Y., Chuman, H., Yoshida, A., Kawano, H., & Kawai, A. (2012). Extraskeletal myxoid chondrosarcoma: A review of 23 patients treated at a single referral center with long-term follow-up. Archives of Orthopaedic and Traumatic Surgery, 132, 1379–1386.CrossRefGoogle Scholar
  4. Oliveira, A. M., Sebo, T. J., McGrory, J. E., Gaffey, T. A., Rock, M. G., & Nascimento, A. G. (2000). Extraskeletal myxoid chondrosarcoma: A clinicopathologic, immunohistochemical, and ploidy analysis of 23 cases. Modern Pathology, 13, 900–908.PubMedCrossRefGoogle Scholar
  5. Suster, S., & Moran, C. A. (1997). Malignant cartilaginous tumors of the mediastinum: Clinicopathological study of six cases presenting as extraskeletal soft tissue masses. Human Pathology, 28, 588–594.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.MD Anderson Cancer CenterHoustonUSA