Abstract
Rhabdomyosarcoma, the most common soft tissue sarcoma noted in childhood, requires multimodality treatment, including chemotherapy, surgical resection, and/or radiation therapy. The majority of the patients with localized rhabdomyosarcoma can be cured; however, the long-term outcomes in patients with metastatic rhabdomyosarcoma remain poor. The standard chemotherapy regimen for patients with rhabdomyosarcoma is the combination of vincristine, actinomycin, and cyclophosphamide/ifosfamide. In recent clinical trials, modifications of the standard chemotherapy protocol have shown improvements in the outcomes in patients with rhabdomyosarcoma. In various type of malignancies, new treatments, such as molecular targeted drugs and immunotherapies, have shown superior clinical outcomes compared to those of standard treatments. Therefore, it is necessary to assess the benefits of these treatments in patients with rhabdomyosarcoma. Moreover, recent basic and clinical studies on rhabdomyosarcoma have reported promising therapeutic targets and novel therapeutic approaches. This article reviews the recent challenges and advances in the management of rhabdomyosarcoma.
Highlights
Rhabdomyosarcoma (RMS) arises from immature cells with the ability to differentiate into skeletal muscle cells in the future
The disease control rates in the ID, IE, and VM group were higher than those in the topotecan, TC, and irinotecan group. These results indicated that the addition of topotecan or irinotecan to the chemotherapy regimen had no benefit for the management of RMS
This study indicated that the addition of maintenance chemotherapy to standard chemotherapy can improve the outcome in patients with high-risk RMS
Summary
Rhabdomyosarcoma (RMS) arises from immature cells with the ability to differentiate into skeletal muscle cells in the future. 60% of the patients with ARMS express. Previous studies have reported that PAX-FOXO1 fusion proteins have oncogenic potential and function as dominant oncogenes in promoting tumorigenesis in fusion-positive RMS [7,8]. Cancers 2020, 12, 1758 for PAX3-FOXO1 or PAX7-FOXO1 significantly correlated with worse event-free survival (EFS) and overall survival (OS) [11]. Based on these reports, PAX-FOXO1 may be considered a strong potential therapeutic target and biomarker predicting prognosis in RMS patients with PAX-FOXO1 translocation. There is a need to develop systemic treatments, which have better oncological and functional outcomes with long-term safety, for patients with RMS. The recent challenges and advancements in the treatment of RMS are discussed
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