Managing axial bone sarcomas in childhood. Issue 7 (3rd July 2021)

• Record Type: Journal Article
• Authors: Meazza, Cristina; Luksch, Roberto; Luzzati, Alessandro
• Is Part Of: Expert review of anticancer therapy. Volume 21:Issue 7(2021)
• Page Start: 747
• Page End: 764
• Publication Date: 2021-07-03
• Subjects: Axial osteosarcoma -- ewing sarcoma -- surgery -- radiotherapy -- chemotherapy -- targeted drugs; Cancer -- Treatment -- Periodicals; 616.99406
• DOI: 10.1080/14737140.2021.1891886
• Languages: English
• ISSNs: 1473-7140

Abstract

ABSTRACT: Introduction : Axial osteosarcoma and Ewing sarcoma are rare, aggressive neoplasms with a worse prognosis than with tumors involving the extremities because they are more likely to be associated with larger tumor volumes, older age, primary metastases, and a poor histological response to chemotherapy. The 5-year OS rates are reportedly in the range of 18–41% for axial osteosarcoma, and 46–64% for Ewing sarcoma. Area covered : The treatment of axial bone tumors is the same as for extremity bone tumors, and includes chemotherapy, surgery and/or radiotherapy. Expert opinion : Local treatment of axial tumors is particularly difficult due to their proximity to neurological and vascular structures, which often makes extensive and en bloc resections impossible without causing significant morbidity. The incidence of local relapse is consequently high, and this is the main issue in the treatment of these tumors. Radiotherapy is an option in the case of surgical resections with close or positive margins, as well as for inoperable tumors. Delivering high doses of RT to the spinal cord can be dangerous. Given the complexity and rarity of these tumors, it is essential for this subset of patients to be treated at selected reference institutions with specific expertise and multidisciplinary skills.