title: The impact of local control in the treatment of type II/III pleuropulmonary blastoma. Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS).
abstract: OBJECTIVE
  This study aims at examining the potential survival benefits of primary versus secondary surgery in the management of children diagnosed with pleuropulmonary blastoma (PPB) type II/III.
  
  
  METHODS
  Disease characteristics, treatment, and survival of 29 children with localized PPB type II/III, treated in six prospective Cooperative Weichteilsarkom Studiengruppe (CWS) trials, were reviewed retrospectively.
  
  
  RESULTS
  Five year event free survival (EFS) and overall survival (OS) of children treated according to CWS protocols was 72%. Patients with tumors ≤10 cm had a 5 year OS of 91% versus 57% in patients with tumors >10 cm (P = 0.025). Five year OS of patients with macroscopically incomplete upfront resections was 44% as opposed to 68% in patients with delayed/secondary microscopically or macroscopically complete resection after an initial biopsy (P = 0.476). Ten patients died of disease, one patient died of second malignancy. Tumor size and complete tumor resection at any time were significant prognostic factors (P = 0.025/0.003) for EFS. EFS for microscopically complete, microscopically incomplete, and macroscopically incomplete resection at any time was 91%, 90%, and 25%, respectively (P = 0.01).
  
  
  CONCLUSIONS
  Primary or secondary microscopically/macroscopically complete tumor resections in combination with chemotherapy correlates with long term survival in children with PPB. J. Surg. Oncol. 2017;115:164-172. © 2017 Wiley Periodicals, Inc.
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Stuttgart, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Sparber-Sauer
authors.affiliation: Department of Pediatric Surgery, University Childreńs Hospital, Marburg, Germany.
authors.forename: Guido
authors.initials: G
authors.lastname: Seitz
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Stuttgart, Germany.|Kinder- und Jugendarztpraxis Dr. Konstatopoulos, Munich, Germany.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Kirsch
authors.affiliation: Institute of Paidopathology, University of Kiel, Kiel, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Vokuhl
authors.affiliation: Institute of Paidopathology, University of Kiel, Kiel, Germany.
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Stuttgart, Germany.
authors.forename: Tobias M
authors.initials: TM
authors.lastname: Dantonello
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Stuttgart, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Scheer
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Institute of Radiology, Stuttgart, Germany.
authors.forename: Thekla
authors.initials: T
authors.lastname: von Kalle
authors.affiliation: Department of Women's and Children's Health, Uppsala University, Uppsala, Sweden.
authors.forename: Gustaf
authors.initials: G
authors.lastname: Ljungman
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Stuttgart, Germany.|Department of Pediatric Hematology and Oncology, University of Muenster, Muenster, Germany.
authors.forename: Stefan S
authors.initials: SS
authors.lastname: Bielack
authors.affiliation: University of Frankfurt, Hospital for Children and Adolescents, Frankfurt/M., Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Klingebiel
authors.affiliation: Department of Pediatric Surgery and Urology, University Childreńs Hospital, Tuebingen, Germany.
authors.forename: Joerg
authors.initials: J
authors.lastname: Fuchs
authors.affiliation: Klinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Stuttgart, Germany.|Department of Pediatric Hematology and Oncology, University Childreńs Hospital, Tuebingen, Germany.
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D018202:Pulmonary Blastoma / Q000473:pathology / Q000628:therapy*; D015996:Survival Rate
keywords: CWS Studiengruppe; Pediatric oncology; pleuropulmonary blastoma; surgery; thoracic tumor