title: Benefit of aggressive multimodality therapy with autologous stem cell support for intra-abdominal desmoplastic small round cell tumor.
abstract: A case is described of a 19-year-old man with poor prognosis intra-abdominal desmoplastic small round cell tumor, who achieved a durable complete remission after aggressive multimodality treatment. He was diagnosed with a large peri-rectal mass and bulky metastases to the peritoneum, omentum and liver. He achieved a partial response to six courses of chemotherapy with VAC (vincristine, adriamycin, actinomycin, cyclophosphamide) and VIP (VP-16, ifosfamide, cisplatin). This was followed by resection of the omentum and cryotherapy to six of the liver lesions. He then achieved a complete response following high-dose busulfan 16 mg/kg and melphalan 140 mg/m2 with autologous stem cell support. Finally, pelvic radiotherapy was given to consolidate the remission. Twenty-six months following his diagnosis, and 19 months after the transplant, he remains well with no clinical or radiologic evidence of recurrent disease. This case report suggests a role for autologous stem cell transplantation as part of multimodality therapy for this unusual and aggressive malignancy.
authors.affiliation: Medical Oncology, Tom Baker Cancer Centre and University of Calgary, Alberta, Canada.
authors.forename: M
authors.initials: M
authors.lastname: Mazuryk
authors.forename: A H
authors.initials: AH
authors.lastname: Paterson
authors.forename: W
authors.initials: W
authors.lastname: Temple
authors.forename: K
authors.initials: K
authors.lastname: Arthur
authors.forename: T
authors.initials: T
authors.lastname: Crabtree
authors.forename: D A
authors.initials: DA
authors.lastname: Stewart
mesh_terms: D000008:Abdominal Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D018714:Radiotherapy, Adjuvant; D014182:Transplantation, Autologous