Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Mediastinal Extraskeletal Osteosarcoma

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


Extraosseous osteogenic sarcoma; Extraosseous osteosarcoma


Extraskeletal osteosarcoma is a malignant osteoid-producing soft tissue neoplasm that by definition is unattached to the bone or periosteum. Contrary to osteosarcoma of the bone which mainly affects adolescents and young adults, extraskeletal osteosarcoma is a disease of middle-aged and older adults which infrequently occurs in patients under the age of 40. Extraskeletal osteosarcoma is a rare malignancy that accounts for only 1–2% of all soft tissue sarcomas and for 2–4% of all osteosarcomas. It most commonly arises in the soft tissues of the lower extremities, especially the thigh but also in the buttocks, shoulder girdle, trunk, and retroperitoneum. Primary mediastinal extraskeletal osteosarcomas have been reported as isolated case reports; to date, less than ten cases have been published in the English literature (Hishida et al. 2009). Several hypotheses as to the histogenesis of these tumors exist...

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References and Further Reading

  1. Catanese, J., Dutcher, J. P., Dorfman, H. D., Andres, D. F., & Wiernik, P. H. (1988). Mediastinal osteosarcoma with extension to lungs in a patient treated for Hodgkin’s disease. Cancer, 62, 2252–2257.PubMedCrossRefGoogle Scholar
  2. Fanburg-Smith, J. C., Bratthauer, G. L., & Miettinen, M. (1999). Osteocalcin and osteonectin immunoreactivity in extraskeletal osteosarcoma: A study of 28 cases. Human Pathology, 30, 32–38.PubMedCrossRefGoogle Scholar
  3. Guillou, L., & Aurias, A. (2010). Soft tissue sarcomas with complex genomic profiles. Virchows Archives, 456, 201–217.CrossRefGoogle Scholar
  4. Hishida, T., Yoshida, J., Nishimura, M., Ishii, G., Nakao, M., & Nagai, K. (2009). Extraskeletal osteosarcoma arising in anterior mediastinum: Brief report with a review of the literature. Journal of Thoracic Oncology, 4, 927–929.PubMedCrossRefGoogle Scholar
  5. Lee, J. S., Fetsch, J. F., Wasdhal, D. A., Lee, B. P., Pritchard, D. J., & Nascimento, A. G. (1995). A review of 40 patients with extraskeletal osteosarcoma. Cancer, 76, 2253–2259.PubMedCrossRefGoogle Scholar
  6. McCarter, M. D., Lewis, J. J., Antonescu, C. R., & Brennan, M. F. (2000). Extraskeletal osteosarcoma: Analysis of outcome of a rare neoplasm. Sarcoma, 4, 119–123.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.MD Anderson Cancer CenterHoustonUSA