BENEFIT OF AGGRESSIVE MULTIMODALITY THERAPY WITH AUTOLOGOUS STEM-CELLSUPPORT FOR INTRAABDOMINAL DESMOPLASTIC SMALL ROUND-CELL TUMOR

A case is described of a 19-year-old man with poor prognosis intra-abd ominal desmoplastic small round cell tumor, who achieved a durable com plete remission after aggressive multimodality treatment. He was diagn osed with a large peri-rectal mass and bulky metastases to the periton eum, omentum and liver. He achieved a partial response to six courses of chemotherapy with VAC (vincristine, adriamycin, actinomycin, cyclop hosphamide) and VIP (VP-16, ifosfamide, cisplatin). This was followed by resection of the omentum and cryotherapy to six of the liver lesion s. He then achieved a complete response following high-dose busulfan 1 6 mg/kg and melphalan 140 mg/m(2) with autologous stem cell support. F inally, pelvic radiotherapy was given to consolidate the remission. Tw enty-six months following his diagnosis, and 19 months after the trans plant, he remains well with no clinical or radiologic evidence of recu rrent disease. This case report suggests a role for autologous stem ce ll transplantation as part of multimodality therapy for this unusual a nd aggressive malignancy.