title: Treatment of children with relapsed soft tissue sarcoma: report of the German CESS/CWS REZ 91 trial.
abstract: BACKGROUND
  The present study was performed to evaluate the possibilities of relapse treatment in patients heavily pretreated for a soft tissue sarcoma.
  
  
  METHODS
  Prospective, multicenter study in 44 soft tissue sarcoma (STS) patients with first relapse. Primary diagnosis was embryonal rhabdomyosarcoma (RME) in 17 patients, alveolar rhabdomyosarcoma (RMA) in 13, primitive neuroectodermal tumor (PNET) in 6, and miscellaneous soft tissue sarcomas in 8 patients. Initial chemotherapy consisted of carboplatin/etoposide combination (150 mg/m2 each, days 1 to 4) followed by local therapy including surgical treatment and, whenever possible, radiotherapy.
  
  
  RESULTS
  In 11/17 patients without primary tumor resection, CR or PR was achieved following the initial two cycles of chemotherapy (61%). The probability of event-free survival (pEFS) for RME patients was 0.41 +/- 0.12 at 5 years, and 0.25 +/- 0.12 for RMA patients. But, in contrast no PNET patient or patient with another soft-tissue sarcoma achieved long-term remission. Additional local radiotherapy significantly (P = 0.002) improved pEFS (3-year estimates of 0.23 +/- 0.2 vs. 0.1 +/- 0.1 in patients without radiotherapy).
  
  
  CONCLUSIONS
  In patients with RME, relapse treatment employing a carboplatin/etoposide combination may induce a second remission in approximately 40% of patients. Surgical excision and additional local radiotherapy seem to be essential to maintain a stable remission. In patients with RMA or PNET, however, this treatment strategy is of no long-term benefit.
authors.affiliation: Children's University Hospital, Tübingen, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Klingebiel
authors.forename: U
authors.initials: U
authors.lastname: Pertl
authors.forename: C F
authors.initials: CF
authors.lastname: Hess
authors.forename: H
authors.initials: H
authors.lastname: Jürgens
authors.forename: E
authors.initials: E
authors.lastname: Koscielniak
authors.forename: R
authors.initials: R
authors.lastname: Pötter
authors.forename: R
authors.initials: R
authors.lastname: van Heek-Romanowski
authors.forename: R
authors.initials: R
authors.lastname: Rossi
authors.forename: C
authors.initials: C
authors.lastname: Schött
authors.forename: H J
authors.initials: HJ
authors.lastname: Spaar
authors.forename: U
authors.initials: U
authors.lastname: Willnow
authors.forename: J
authors.initials: J
authors.lastname: Treuner
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004334:Drug Administration Schedule; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005858:Germany; D006801:Humans; D006979:Hyperthermia, Induced; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D017599:Neuroectodermal Tumors / Q000628:therapy; D011446:Prospective Studies; D012008:Recurrence; D012208:Rhabdomyosarcoma / Q000628:therapy; D012509:Sarcoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D018906:Antineoplastic Agents, Alkylating; D000972:Antineoplastic Agents, Phytogenic; D005047:Etoposide; D016190:Carboplatin; D007069:Ifosfamide