title: Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV, 1978-1997: report from the Children's Oncology Group.
abstract: OBJECTIVE
  Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV.
  
  
  METHODS
  We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail.
  
  
  RESULTS
  The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ("better" sites, 55%), paranasal sinus and infratemporal-pterygopalatine area ("worse" sites, 42%), and other (3%). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues.
  
  
  RESULTS
  The estimated 10-year failure-free survival (FFS) rate was 32% (95% confidence interval [CI]: 22%, 42%). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55%), local (12%), CNS extension (8%), mixed (6%), and uncertain (18%). Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06).
  
  
  CONCLUSIONS
  Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.
authors.affiliation: Children's Cancer Hospital, University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA. rbraney@austin.rr.com
authors.forename: Beverly
authors.initials: B
authors.lastname: Raney
authors.forename: James
authors.initials: J
authors.lastname: Anderson
authors.forename: John
authors.initials: J
authors.lastname: Breneman
authors.forename: Sarah S
authors.initials: SS
authors.lastname: Donaldson
authors.forename: Winston
authors.initials: W
authors.lastname: Huh
authors.forename: Harold
authors.initials: H
authors.lastname: Maurer
authors.forename: Jeff
authors.initials: J
authors.lastname: Michalski
authors.forename: Stephen
authors.initials: S
authors.lastname: Qualman
authors.forename: Fred
authors.initials: F
authors.lastname: Ullrich
authors.forename: Moody
authors.initials: M
authors.lastname: Wharam
authors.forename: William
authors.initials: W
authors.lastname: Meyer
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D009362:Neoplasm Metastasis / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome