Desmoplastic small round cell tumor (DSRCT) is a very aggressive type of cancer. Currently at Memorial Sloan-Kettering Cancer Center, patients with newly diagnosed DSRCT start treatment with chemotherapy called P6. They then have surgery if it is possible, receive more P6 chemotherapy, and then have radiation therapy. In many patients, however, the cancer will come back or recur after treatment is finished.
Irinotecan, temozolomide, and bevacizumab have been added to the treatment of patients with other types of cancer, but they have not been studied in patients with DSRCT. Irinotecan is used to treat colorectal cancer. Temozolomide is used to treat brain tumors and sometimes melanoma. The combination of irinotecan and temozolomide has been shown to have activity in sarcomas that arise in younger patients. Bevacizumab is used to treat cancers of the colon, lung, breast, kidney, and brain, and works by inhibiting vascular endothelial growth factor (VEGF), which promotes the growth of blood vessels that feed tumors.
The use of these drugs in DSRCT is considered investigational. In this study, researchers want to observe the safety and effectiveness of adding these three drugs to P6 treatment in patients newly diagnosed with DSRCT.