title: Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children's Oncology Group.
abstract: OBJECTIVE
  To determine local control according to clinical variables for patients with intermediate-risk rhabdomyosarcoma (RMS) treated on Children's Oncology Group protocol D9803.
  
  
  METHODS
  Of 702 patients enrolled, we analyzed 423 patients with central pathology-confirmed group III embryonal (n=280) or alveolar (group III, n=102; group I-II, n=41) RMS. Median age was 5 years. Patients received 42 weeks of VAC (vincristine, dactinomycin, cyclophosphamide) or VAC alternating with VTC (T = topotecan). Local therapy with 50.4 Gy radiation therapy with or without delayed primary excision began at week 12 for group III patients. Patients with group I/II alveolar RMS received 36-41.4 Gy. Local failure (LF) was defined as local progression as a first event with or without concurrent regional or distant failure.
  
  
  RESULTS
  At a median follow-up of 6.6 years, patients with clinical group I/II alveolar RMS had a 5-year event-free survival rate of 69% and LF of 10%. Among patients with group III RMS, 5-year event-free survival and LF rates were 70% and 19%, respectively. Local failure rates did not differ by histology, nodal status, or primary site, though there was a trend for increased LF for retroperitoneal (RP) tumors (P=.12). Tumors ≥5 cm were more likely to fail locally than tumors <5 cm (25% vs 10%, P=.0004). Almost all (98%) RP tumors were ≥5 cm, with no difference in LF by site when the analysis was restricted to tumors ≥5 cm (P=.86).
  
  
  CONCLUSIONS
  Local control was excellent for clinical group I/II alveolar RMS. Local failure constituted 63% of initial events in clinical group III patients and did not vary by histology or nodal status. The trend for higher LF in RP tumors was related to tumor size. There has been no clear change in local control over RMS studies, including IRS-III and IRS-IV. Novel approaches are warranted for larger tumors (≥5 cm).
authors.affiliation: Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York. Electronic address: woldens@mskcc.org.
authors.forename: Suzanne L
authors.initials: SL
authors.lastname: Wolden
authors.affiliation: Department of Preventive and Societal Medicine, Nebraska Medical Center, Omaha, Nebraska.
authors.forename: Elizabeth R
authors.initials: ER
authors.lastname: Lyden
authors.affiliation: Department of Pediatric and Adolescent Medicine, Mayo Clinic and Foundation, Rochester, Minnesota.
authors.forename: Carola A
authors.initials: CA
authors.lastname: Arndt
authors.affiliation: Division of Hematology/Oncology, Seattle Children's Hospital, Fred Hutchinson Cancer Research Center, University of Washington, Seattle, Washington.
authors.forename: Douglas S
authors.initials: DS
authors.lastname: Hawkins
authors.affiliation: Frontier Science and Technology Research Foundation, Madison, Wisconsin.
authors.forename: James R
authors.initials: JR
authors.lastname: Anderson
authors.affiliation: Department of Surgery, East Carolina University, Greenville, North Carolina.
authors.forename: David A
authors.initials: DA
authors.lastname: Rodeberg
authors.affiliation: Department of Orthopedic Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Carol D
authors.initials: CD
authors.lastname: Morris
authors.affiliation: Department of Radiation Oncology, Stanford University School of Medicine, Stanford, California.
authors.forename: Sarah S
authors.initials: SS
authors.lastname: Donaldson
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008577:Meningeal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D011879:Radiotherapy Dosage; D012186:Retroperitoneal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D018232:Rhabdomyosarcoma, Alveolar / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D018233:Rhabdomyosarcoma, Embryonal / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D019772:Topotecan / Q000008:administration & dosage; D017211:Treatment Failure; D047368:Tumor Burden; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D019772:Topotecan; D003520:Cyclophosphamide