title: Relapse and outcome patterns of patients with central nervous system mixed malignant germ cell tumors treated without irradiation: Findings from the third international central nervous system (CNS) germ cell tumor (GCT) study.
abstract: OBJECTIVE
  To evaluate patterns of relapse and outcome in patients newly diagnosed with CNS Mixed Malignant GCT (MMGCT) treated initially with chemotherapy alone.
  
  
  METHODS
  A retrospective chart review was conducted using all 25 patients enrolled on the International CNS GCT Study III, with at least 7 years follow-up for all surviving patients.
  
  
  RESULTS
  Thirteen patients at diagnosis had CNS MMGCT by pathology and tumor markers (n = 11), or tumor markers alone (n = 2). Twelve received chemotherapy alone, one additionally receiving focal irradiation prior to relapse. Six patients (46%) relapsed (mean of 30.5 months; range 6-59 months), two beyond and four within the primary site alone. Three patients relapsed early (6-23 months from diagnosis), two with alpha-fetoprotein elevations and one without tumor markers assessed; all three expired of progressive disease at 2-10 months following initial relapse. Three patients relapsed late (37-59 months) without AFP elevations, one with pathologically pure germinoma, two with mild beta-human chorionic gonadotropin elevations; these patients survive disease-free at 86+, 94+, and 126+ months following additional treatment.
  
  
  CONCLUSIONS
  Patients with CNS MMGCT relapsing following chemotherapy alone display two distinct patterns of recurrence and outcome; patients relapsing early possess MMGCT elements and have a dismal prognosis, while patients relapsing late do so with pure germinomatous elements and have an excellent outcome. Current cooperative group studies utilizing more localized fields of irradiation should monitor closely the patterns of relapse and outcome; late recurrences with germinomatous elements might be avoided by initial use of low-dose larger field irradiation in select patients.
authors.affiliation: Children's Center for Cancer & Blood Diseases, Children's Hospital Los Angeles, Los Angeles, California.|Hofstra North Shore-LIJ School of Medicine, Hempstead, New York, New York.
authors.forename: Rachel
authors.initials: R
authors.lastname: Pruitt
authors.affiliation: Neuro-oncology Program, GRAACC Institute of Pediatric Oncology, Sao Paolo, Brazil.
authors.forename: Nasjla S
authors.initials: NS
authors.lastname: DaSilva
authors.affiliation: Neuro-oncology Program, GRAACC Institute of Pediatric Oncology, Sao Paolo, Brazil.
authors.forename: Andrea
authors.initials: A
authors.lastname: Cappellano
authors.affiliation: Children's Center for Cancer & Blood Diseases, Children's Hospital Los Angeles, Los Angeles, California.|North Middlesex University Hospital, London, UK.
authors.forename: Clara
authors.initials: C
authors.lastname: Belessiotis
authors.affiliation: Neuro-Oncology Program, Fundacion para la Lucha contra Enfermedades Neurologicas de la Infancia, Buenos Aires, Argentina.
authors.forename: Blanca
authors.initials: B
authors.lastname: Diez
authors.affiliation: New York University Medical Center, New York, New York.
authors.forename: Sharon
authors.initials: S
authors.lastname: Gardner
authors.affiliation: New York University Medical Center, New York, New York.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Allen
authors.affiliation: Pediatric Hematology/Oncology, Winthrop-University Hospital, New York, New York.
authors.forename: Mark
authors.initials: M
authors.lastname: Weinblatt
authors.affiliation: Pediatric Oncology, Princess Margaret Hospital, Perth, Australia.
authors.forename: Nicholas
authors.initials: N
authors.lastname: Gottardo
authors.affiliation: Children's Center for Cancer & Blood Diseases, Children's Hospital Los Angeles, Los Angeles, California.
authors.forename: Girish
authors.initials: G
authors.lastname: Dhall
authors.affiliation: Nationwide Children's Hospital and the Ohio State University, Columbus, Ohio.
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Finlay
mesh_terms: D000293:Adolescent; D016543:Central Nervous System Neoplasms / Q000401:mortality* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D017326:Clinical Trials, Phase III as Topic; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000401:mortality* / Q000628:therapy; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate
keywords: CNS germ cell tumors; chemotherapy; relapse