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A desmoplastic small-round-cell tumor (DSRCT) is a rare, aggressive neoplasm that develops mostly in the abdominal cavity in children and young adults. We present a case of a 19-year-old male with right upper quadrant discomfort for 3 months. On abdominal computerized tomography, multiple huge and demarcated masses were found in the liver, retroperitoneal lymph nodes, and peritoneal and retroperitoneal cavities. Fine needle aspiration biopsy of the hepatic mass was performed and DSRCT was diagnosed by hematoxylin and eosin staining and immunohistochemical analysis. He was treated initially with high-dose systemic chemotherapy (alternating schedules of cyclophosphamide, vincristine, doxorubicin, ifosfamide, and etoposide), underwent two debulking surgeries and pelvic irradiation between systemic chemotherapy schedules, and achieved complete remission after the 15 months of treatment duration. We report this case to emphasize the importance of aggressive local treatment modalities as well as high-dose systemic chemotherapy for treatment of DSRCT even with initially unresectable or extensively metastatic presentation. (Korean J Med 2012;83:378-384)

Desmoplastic Small Round Cell Tumor Achieved Complete Remission after High-Dose Chemotherapy with Aggressive Local Treatment Modalities