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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. [ABSTRACT FROM AUTHOR]

Preservation of the sciatic nerve is difficult in cases of highly malignant soft tissue tumors closely surrounding the nerve. Herein, we present the first case of preservation of this nerve by combining an in-situ preparation technique (ISP; a technique enabling the preparation of neurovascular bundles without contamination by tumor cells) with intensive concurrent neoadjuvant chemo-radiotherapy with hyperthermia (RHC; radio-hyperthermo-chemotherapy). A 62-year-old man presented with a soft tissue mass in the right thigh and was diagnosed with undifferentiated pleomorphic sarcoma. The tumor arose in the multi-compartment areas of the posterior thigh muscles and was closely intertwined with the sciatic nerve. As preoperative therapy, RHC was performed for surgical down-staging and the tumor partially responded. Afterwards, wide resection of the tumor with preservation of the sciatic nerve using ISP was performed. Following the surgery, there has not been recurrence in the affected site and the functional outcomes of the lower extremity achieved 80% in the Musculoskeletal Tumor Society score. The patient is still alive with disease five years postoperatively. This is the first case in which ISP and RHC procedures were combined for the preservation of the neurovascular structure. Further study is needed for the validation of the feasibility of this method. [ABSTRACT FROM AUTHOR]