title: Primary metastatic Ewing's family tumors: results of the Italian Sarcoma Group and Scandinavian Sarcoma Group ISG/SSG IV Study including myeloablative chemotherapy and total-lung irradiation.
abstract: BACKGROUND
  The Italian Sarcoma Group and the Scandinavian Sarcoma Group designed a joint study to improve the prognosis for patients with Ewing's family tumors and synchronous metastatic disease limited to the lungs, or the pleura, or a single bone.
  
  
  METHODS
  The study was opened in 1999 and closed to the enrollment in 2008. The program consisted of intensive five-drug combination chemotherapy, surgery and/or radiotherapy as local treatment, and consolidation treatment with high-dose busulfan/melphalan plus autologous stem cell rescue and total-lung irradiation.
  
  
  RESULTS
  During the study period, 102 consecutive patients were enrolled. The median follow-up was 62 months (range 24-124). The 5-year event-free survival probability was 0.43 [standard deviation (SD) = 0.05] and the 5-year overall survival probability was 0.52 (SD = 0.052). Unfavorable prognostic factors emerging on multivariate analysis were a poor histological/radiological response at the site of the primary tumor [relative risk (RR) = 3.4], and incomplete radiological remission of lung metastases after primary chemotherapy (RR = 2.6). One toxic death and one secondary leukemia were recorded.
  
  
  CONCLUSIONS
  This intensive approach is feasible and long-term survival is achievable in ∼50% of patients. New treatment approaches are warranted for patients responding poorly to primary chemotherapy.
authors: Department of Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan. Electronic address: roberto.luksch@istitutotumori.mi.it.
authors: R
authors: R
authors: Luksch
authors: Department of Oncology, S. Maria delle Croci Hospital, Ravenna, Italy.
authors: A
authors: A
authors: Tienghi
authors: Department of Oncology, Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway.
authors: K Sundby
authors: KS
authors: Hall
authors: Department of Pediatric Onco-Hematology, Regina Margherita Children's Hospital, Turin.
authors: F
authors: F
authors: Fagioli
authors: Department of Musculoskeletal Oncology, Experimental Oncology Division, Istituto Ortopedico Rizzoli, Bologna.
authors: P
authors: P
authors: Picci
authors: Department of Radiotherapy, University of Bologna, Policlinico Sant'Orsola, Bologna.
authors: E
authors: E
authors: Barbieri
authors: Department of Radiotherapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: L
authors: L
authors: Gandola
authors: Skane Onkologiska Klinik, Lund University Hospital and Lund University, Lund, Sweden.
authors: M
authors: M
authors: Eriksson
authors: Department of Musculoskeletal Oncology, Division of Surgery, Istituto Ortopedico Rizzoli, Bologna.
authors: P
authors: P
authors: Ruggieri
authors: Oncologic/Orthopedic Surgery, Istituto Ortopedico G. Pini, Milan, Italy.
authors: P
authors: P
authors: Daolio
authors: Department of Oncology and Radiotherapy, Turku University Hospital, Turku, Finland.
authors: P
authors: P
authors: Lindholm
authors: Department of Pediatric Oncology, University of Bologna, Policlinico Sant'Orsola, Bologna.
authors: A
authors: A
authors: Prete
authors: Department of Pediatric Oncology, University of Padua, Padua.
authors: G
authors: G
authors: Bisogno
authors: Department of Pediatric Onco-Hematology, Ospedale Meyer, Florence.
authors: A
authors: A
authors: Tamburini
authors: Department of Medical Oncology, Institute for Cancer Research and Treatment, Candiolo.
authors: G
authors: G
authors: Grignani
authors: Department of Musculoskeletal Oncology, Division of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors: M E
authors: ME
authors: Abate
authors: Department of Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan.
authors: M
authors: M
authors: Podda
authors: Department of Oncology, Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway.
authors: S
authors: S
authors: Smeland
authors: Department of Musculoskeletal Oncology, Division of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors: S
authors: S
authors: Ferrari
mesh_terms: D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000556:secondary* / Q000628:therapy; D002066:Busulfan / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D008175:Lung Neoplasms / Q000532:radiotherapy / Q000556:secondary* / Q000628:therapy; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D019653:Myeloablative Agonists / Q000627:therapeutic use*; D009362:Neoplasm Metastasis / Q000188:drug therapy / Q000532:radiotherapy; D011379:Prognosis; D012512:Sarcoma, Ewing / Q000188:drug therapy / Q000532:radiotherapy / Q000556:secondary / Q000628:therapy*; D033581:Stem Cell Transplantation; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D019653:Myeloablative Agonists; D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D002066:Busulfan; D008558:Melphalan; D007069:Ifosfamide