Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Rhabdomyosarcoma, Mediastinal

  • Annikka Weissferdt
  • Cesar A. Moran
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-66796-6_47

Definition

Rhabdomyosarcoma is the most common soft tissue sarcoma in children accounting for 4–8% of all malignancies in children under the age of 15 years. It is an uncommon neoplasm in adults in whom it merely accounts for 2–5% of all soft tissue sarcomas. These tumors show rhabdomyoblastic differentiation and most commonly occur in the extremities, head and neck area, or genitourinary tract. Rhabdomyosarcomas arising in the mediastinum are rare and most often a component of a teratoma, non-teratomatous germ cell tumor, or thymic carcinosarcoma. Pure mediastinal rhabdomyosarcomas have only sporadically been reported in the literature as case reports and only a single larger series of four cases (Suster et al. 1994). The tumors can be subdivided into three large categories based on histological appearance and genetic features: embryonal, alveolar, and pleomorphic rhabdomyosarcoma. The embryonal and alveolar types predominate in children, while the pleomorphic variant has a higher...

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

References and Further Reading

  1. Horn, R. C., Jr., & Enterline, H. T. (1958). Rhabdomyosarcoma: A clinicopathological study and classification of 39 cases. Cancer, 11, 181–199.CrossRefPubMedGoogle Scholar
  2. Missiaglia, E., Williamson, D., Chisholm, J., Wirapati, P., Pierron, G., Petel, F., Concordet, J. P., Thway, K., Oberlin, O., Pritchard-Jones, K., Delattre, O., Delorenzi, M., & Shipley, J. (2012). PAX3/FOXO1 fusion gene status is the key prognostic molecular marker in rhabdomyosarcoma and significantly improves current risk stratification. Journal of Clinical Oncology, 30, 1670–1677.CrossRefPubMedGoogle Scholar
  3. Qi, Y., Chang, B., Pang, L., Liu, C., & Li, F. (2011). Solid alveolar rhabdomyosarcoma with spindle-shaped cells and epithelial differentiation of the mediastinum in a 68-year-old man: A case report and literature review. Journal of Cancer Research and Therapeutics, 7, 353–356.CrossRefPubMedGoogle Scholar
  4. Suster, S., Moran, C. A., & Koss, M. N. (1994). Rhabdomyosarcomas of the anterior mediastinum: Report of four cases unassociated with germ cell, teratomatous, or thymic carcinomatous components. Human Pathology, 25, 349–356.CrossRefPubMedGoogle Scholar
  5. Tiffin, N., Williams, R. D., Shipley, J., & Pritchard-Jones, K. (2003). PAX7 expression in embryonal rhabdomyosarcoma suggests an origin in muscle satellite cells. British Journal of Cancer, 89, 327–332.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.MD Anderson Cancer CenterHoustonUSA