Abstract
Desmoplastic small round cell tumor (DSRCT) is a highly aggressive and malignant tumor that typically occurs in the abdomen. The prognosis for this rare type of soft tissue sarcoma remains extremely poor. The article presents the clinical follow-up of a 16 year-old girl with pelvic cavity-origin DSRCT with multiple-organ metastases : regional lymph node metastases, distant metastasis to the lungs, liver, and bone marrow. Current approved treatment protocols of DSRCT do not exist. We attempted to perform chemotherapy on protocols appropriate for the treatment of other small round cell tumors – VAC consolidation therapy; but the tumor has progressed.
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