title: Outcome of patients with localized orbital sarcoma who relapsed following treatment on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III and -IV, 1984-1997: a report from the Children's Oncology Group.
abstract: BACKGROUND
  We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997.
  
  
  METHODS
  Retrospective chart review.
  
  
  RESULTS
  Twenty-four of 188 patients (12.8%) developed local (n = 22) or distant relapse (n = 2) at 0.057-7.05 years (median, 1.58) after enrollment. Ages at study entry were 0.14-17 years (median, 5 years). Initial tumor operations included biopsy (n = 20) or gross resection with microscopic residual (n = 4). Initial tumor diameters were 0.5-7 cm (median, 3). Pathologic subtypes were embryonal rhabdomyosarcoma (ERMS, n = 19), sarcoma not otherwise specified (n = 2), and alveolar RMS, botryoid ERMS, or undifferentiated sarcoma (n = 1 each). Initial treatment included vincristine/dactinomycin (n = 24) including an alkylator (n = 4) and radiotherapy (RT, n = 21). Twenty patients responded, 14 completely, 6 partially. After recurrence, patients underwent orbital exenteration (n = 10), enucleation (2), tumor excision (3), or biopsy (1); 7 had no operation, and 1 had no data. Post-relapse chemotherapy included combinations of etoposide (n = 14 patients), doxorubicin (14), ifosfamide (12), cyclophosphamide (7), and dacarbazine (n = 1). Six patients received RT, including four previously treated and two not irradiated initially. Two patients died; one at 1.79 years after contralateral brain metastasis followed by local recurrence, and another at 2.49 years after multiple local recurrences. Twenty-two patients (91.7%) survived sarcoma-free for 0.04-17 years (median, 6.9) after relapse, and 18 of 22 (82%) were alive ≥5 years after relapse.
  
  
  CONCLUSIONS
  Survival following recurrent localized orbital sarcoma appears likely after vigorous re-treatment given with curative intent.
authors.affiliation: Division of Pediatrics and Children's Cancer Hospital, U.T. M.D. Anderson Cancer Center, Houston, Texas, USA. rbraney@austin.rr.com
authors.forename: Beverly
authors.initials: B
authors.lastname: Raney
authors.forename: Winston
authors.initials: W
authors.lastname: Huh
authors.forename: Douglas
authors.initials: D
authors.lastname: Hawkins
authors.forename: Andrea
authors.initials: A
authors.lastname: Hayes-Jordan
authors.forename: Lynn
authors.initials: L
authors.lastname: Million
authors.forename: David
authors.initials: D
authors.lastname: Rodeberg
authors.forename: Lisa
authors.initials: L
authors.lastname: Teot
authors.forename: James
authors.initials: J
authors.lastname: Anderson
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D017326:Clinical Trials, Phase III as Topic; D017327:Clinical Trials, Phase IV as Topic; D003131:Combined Modality Therapy; D005260:Female; D049690:History, Ancient; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology; D009918:Orbital Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011878:Radiotherapy; D058028:Research Report; D012189:Retrospective Studies; D012509:Sarcoma / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome