title: Intensive combined modality therapy of small round cell and undifferentiated sarcomas in children and young adults: local control and patterns of failure.
abstract: Seventy-five patients (ages 4-35 years) with the following small round cell tumors and undifferentiated sarcoma were treated at the National Cancer Institute: Ewing's sarcoma (n = 32), peripheral neuroepithelioma (n = 14), rhabdomyosarcoma (n = 24), undifferentiated sarcoma (n = 5). Most patients had poor prognostic features including 36 (48%) with metastatic disease, and 42 (56%) with central (truncal) tumors (22 in the pelvis). Treatment included 5 cycles of intensive induction chemotherapy with vincristine, cyclophosphamide and adriamycin, plus aggressive local radiation therapy using simulation and computerized treatment planning for all patients. Thereafter, complete clinical responses were consolidated with intensive chemotherapy, total body irradiation and autologous bone marrow transplantation. There were three local only failures, 10 local plus distant failures, 36 distant only failures, 3 treatment-related deaths, and one intercurrent death. Overall actuarial survival and event-free survival at 4 years are 49 and 29%, respectively. Actuarial freedom from local progression was seen in 74% of patients at 4 years, quite remarkable considering the bulk and location of most of these tumors. Without aggressive surgery, many of these high risk patients had satisfactory outcomes, but better systemic treatments are still needed.
authors.affiliation: Radiation Oncology Branch, National Cancer Institute, Bethesda, Maryland 20892.
authors.forename: J L
authors.initials: JL
authors.lastname: Bader
authors.forename: M E
authors.initials: ME
authors.lastname: Horowitz
authors.forename: R
authors.initials: R
authors.lastname: Dewan
authors.forename: E
authors.initials: E
authors.lastname: Watkins
authors.forename: T J
authors.initials: TJ
authors.lastname: Triche
authors.forename: M
authors.initials: M
authors.lastname: Tsokos
authors.forename: T J
authors.initials: TJ
authors.lastname: Kinsella
authors.forename: J S
authors.initials: JS
authors.lastname: Miser
authors.forename: S M
authors.initials: SM
authors.lastname: Steinberg
authors.forename: E
authors.initials: E
authors.lastname: Glatstein
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D009367:Neoplasm Staging; D011879:Radiotherapy Dosage; D012509:Sarcoma / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*