Patients with DSRCT require aggressive multimodality therapy. Current treatment protocols with neoadjuvant chemotherapy, debulking of >90% of the tumor, and radiation therapy have been reported to prolong life but rarely achieve cure. Chemotherapeutic agents with known activity in DSRCT are very similar to those active in EWS. Both tumors share an EWS fusion protein and may also share molecular mechanisms that facilitate proliferation and survival pathways. Say no to plagiarism. Get a tailor-made essay on "Why Violent Video Games Shouldn't Be Banned"? Get an original essay Alkylating agents such as cyclophosphamide and ifosfamide are important components of the therapy. Currently, a well-known treatment scheme was reported by Kushner et al.19 who described the results in 12 DSRCT patients. This intensive alkylating therapy used cyclophosphamide, doxorubicin, vincristine alternating with ifosfamide and etoposide. Its combination with other treatment modalities such as surgery, radiotherapy, autologous stem cell recovery, or the combination of all of the above has been used. The median survival time was 19 months. For those who achieved a complete response, the median follow-up in this series was 22 months. The toxicity of this regimen can be substantial and often results in hospitalization not only for chemotherapy, but also for fevers associated with myelosuppression and mucositis. A more tolerable alternative outpatient regimen has more recently been used for DSRCT, as documented in a case report.20 In this report, neoadjuvant chemotherapy included vincristine, ifosfamide, dexrazoxane/doxorubicin, and etoposide. Continuous hyperthermic peritoneal perfusion HIPEC with cisplatin was administered after extensive cytoreductive surgery. This was followed by irinotecan+temozolomide every 2 months and then 30Gy abdominal radiotherapy with concurrent temozolomide. A total of 12 cycles of irinotecan and temozolomide were administered which allowed a disease-free interval of almost 2 years. It allowed regular school attendance and recreational activities were possible with an excellent quality of life. It is now accepted that aggressive cytoreductive surgery has a primary role in achieving prolonged survival of other malignancies involving the peritoneum.21–23 In the DSRCT, La Quaglia and Brennan6 reported that the 3-year overall survival was 58% in patients with gross total resection compared to 0% in the non-resection cohort. Other treatment modalities such as CHPP, also called hyperthermic intrapertitoneal chemotherapy (HIPEC), have been found to improve outcomes in carcinoma involving the peritoneum. Please note: this is just a sample. Get a custom paper from our expert writers now. Customize Essay23-26 Recently, Hayes-Jordan and colleagues were the first to report the combined use of cytoreductive surgery and HIPEC in 2 children with DSCRT. Both children received neoadjuvant chemotherapy, followed by cytoreductive surgery and intraoperative HIPEC with cisplatin. At the time of publication, both patients had no evidence of disease 6 and 10 months after surgery.27 A phase I study using HIPEC is currently underway at MD Anderson Cancer Center.8 A more recent series reports a median 3-year survival of 71% with HIPEC and 29% in those receiving chemotherapy and radiotherapy alone.