title: Favorable outcome of Ewing sarcoma family tumors to multiagent intensive preoperative chemotherapy: a single institution experience.
abstract: BACKGROUND
  Aim of our study was to evaluate the efficacy of multiagent intensive preoperative chemotherapy in patients with Ewing sarcoma family tumors (ESFT), in order to succeed a better percentage of necrosis before surgical resection.
  
  
  METHODS
  Eighteen patients with ESFT were treated with the same multiagent intensive preoperative protocol. 5/18 patients had bone Ewings sarcoma (EWS) and 13/18 had peripheral primitive neuroectodermal tumor (PNET). None had metastases at diagnosis. Chemotherapy consisted of 5 or 6 cycles with vincristine, cisplatin, cyclophosphamide, and Adriamycin, followed by 12 cycles of vincristine, cyclophosphamide, and actinomycin-D. Five patients with EWS underwent total resection after 5-6 cycles of preoperative chemotherapy and prosthetic replacement was performed in two of them. In 3/13 patients with PNET the tumor was resected at diagnosis and in 1/13 after 5 cycles of chemotherapy, while 9/13 patients received chemotherapy only and/or radiotherapy.
  
  
  RESULTS
  In patients with EWS, the histologic specimens of the resected tumors showed that tissue necrosis was 100% in four patients and 95% in one patient. The good histologic response reflects the effectiveness of this regimen in all ESFT. No patient had topical recurrence or developed metastatic disease during follow-up period (2-13 years, mean time 7.4 years). All patients had the scheduled cycles without delays or dose reductions. There were no major side effects of chemotherapy.
  
  
  CONCLUSIONS
  The intensive chemotherapy schedule, comprising of 5-6 cycles preoperatively, seems to maximize the percentage of tumor necrosis, thus improving outcome. Our study implies that this combined therapy may improve the prognosis of ESFT.
authors.affiliation: Hematology-Oncology Unit, First Department of Pediatrics of Athens University, Greece. mmoschov@med.uoa.gr <mmoschov@med.uoa.gr>
authors.forename: Maria
authors.initials: M
authors.lastname: Moschovi
authors.forename: Georgios
authors.initials: G
authors.lastname: Trimis
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Stefanaki
authors.forename: John
authors.initials: J
authors.lastname: Anastasopoulos
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Syriopoulou
authors.forename: Eleutheria
authors.initials: E
authors.lastname: Koultouki
authors.forename: Fotini
authors.initials: F
authors.lastname: Tzortzatou-Stathopoulou
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000235:genetics* / Q000401:mortality / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000188:drug therapy / Q000235:genetics / Q000401:mortality / Q000601:surgery; D011300:Preoperative Care; D011879:Radiotherapy Dosage; D012512:Sarcoma, Ewing / Q000188:drug therapy* / Q000235:genetics* / Q000401:mortality / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D002945:Cisplatin