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...
References and Further Reading
- 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
- 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