title: Topotecan plus cyclophosphamide in adults with relapsed or refractory pediatric-type sarcoma: a retrospective analysis from the German Sarcoma Medical Oncology Group (AIO).
abstract: BACKGROUND
  To assess the efficacy and safety of topotecan and cyclophosphamide (TC) in adult patients with pediatric-type sarcoma subtypes who failed induction chemotherapy.
  
  
  METHODS
  Patients with pediatric sarcoma subtypes, refractory to or relapsed after at least one prior induction chemotherapy, inoperable, ECOG PS 0-2, with measurable, progressive disease (PD), adequate organ functions, who have been treated with TC combination were retrospectively analysed within the AIO and SAREZ/BMBF network.
  
  
  RESULTS
  Thirty-nine patients, median age 28 years (18-58), 14 females, 25 males, have been identified. All patients had received induction treatment according to (inter)national study protocols. Second-line TC was applied in 33 patients (≥3rd-line in 6 patients). Twenty-three patients had refractory disease (evidence of PD during induction chemotherapy); 8 patients experienced an early relapse within 6 months as well as 8 patients after more than 24 months (late relapse). A median of 3 cycles (range, 1-6) had been applied and antitumor activity was: CR 2.6 %, PR 7.9 %, and disease stabilisation (SD) 26.3 %. PR lasted 32.8 months and median duration in patients with SD was 5 months (range, 2.0-14.7). The 3/6-months progression-free rates were 43.2 and 18.9 %.
  
  
  CONCLUSIONS
  Limited activity was seen in adult pts with refractory or relapsed pediatric-type sarcomas with the regimen which has proven activity in pediatric patients. Adults with refractory small cell sarcoma appear to have a similar dismal outcome as seen in pts with common adult-type histologies; however, a subset of patients has achieved long-lasting remissions on TC resulting in long-term survival.
authors.affiliation: Clinic for Internal Medicine II, Hematology, Oncology, Immunology, Catholic Hospital Consortium, Franziskus Hospital Bielefeld, Kiskerstrasse 26, Bielefeld, 33615, Germany. joerg.hartmann@franziskus.de.
authors.forename: Jörg Thomas
authors.initials: JT
authors.lastname: Hartmann
authors.affiliation: Department of Hematology and Oncology, University of Munich Medical Center, München, Germany.
authors.forename: R D
authors.initials: RD
authors.lastname: Issels
authors.affiliation: Department of Hematology and Medical Oncology, University Hospital of Erlangen, Erlangen, Germany.
authors.forename: K San
authors.initials: KS
authors.lastname: Nicolo
authors.affiliation: Department of Hematology, Hemostaseology, Oncology, and Stem Sell Transplantation, Medical University of Hannover, Hannover, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Grünwald
authors.affiliation: Department of Hematology and Oncology, Medical Center of Bremen-Mitte gGmbH, Bremen, Germany.
authors.forename: B
authors.initials: B
authors.lastname: Hertenstein
authors.affiliation: Department of Hematology, Medical Oncology and Pneumonology, University Hospital of Mainz, Mainz, Germany.
authors.forename: E
authors.initials: E
authors.lastname: Papesch
authors.affiliation: Department of Hematology and Medical Oncology, University Hospital of Erlangen, Erlangen, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Krause
authors.affiliation: Department of Hematology, Oncology and Tumor Immunology, Charité - University Hospital Berlin, Campus Virchow-Klinikum, Berlin, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Sturm
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000188:drug therapy* / Q000401:mortality; D016019:Survival Analysis; D019772:Topotecan / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D019772:Topotecan; D003520:Cyclophosphamide
keywords: Chemotherapy; Cyclophosphamide; Pediatric sarcoma; Refractory disease; Topotecan