title: Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience.
abstract: OBJECTIVE
  To investigate the characteristics of patients with hepatoblastoma and low serum alpha-fetoprotein (AFP) at diagnosis.
  
  
  METHODS
  Inclusion of all 21 patients accrued onto SIOPEL trials, whose serum AFP was <100ng/ml at diagnosis. Slides of all 15 patients with available histological material were centrally reviewed.
  
  
  RESULTS
  Median age: 10 months. Disease extension at diagnosis: PRETEXT group: II (3 patients), III (10 patients) and IV (8 patients). Extra-hepatic extension: 8 patients. Multifocal tumour: 8 patients. Histology at review: wholly epithelial subtype: 11/15 patients including nine with a small-cell undifferentiated histology.
  
  
  RESULTS
  only 9 patients achieved a partial response and 16 died. Median survival: 4.4 months. Two-year overall survival: 24% (confidence interval 10-45%).
  
  
  CONCLUSIONS
  This study clearly identifies patients with hepatoblastoma and low serum AFP at diagnosis as a high-risk subgroup with extensive disease at diagnosis, poor response to chemotherapy and a poor outcome.
authors.affiliation: Department of Paediatric Oncology, Institut Gustave Roussy, 39, Rue Camille Desmoulins, 94805 Villejuif, France.
authors.forename: Maretta
authors.initials: M
authors.lastname: De Ioris
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugieres
authors.forename: Arthur
authors.initials: A
authors.lastname: Zimmermann
authors.forename: Jean
authors.initials: J
authors.lastname: Keeling
authors.forename: Penelope
authors.initials: P
authors.lastname: Brock
authors.forename: Rudolf
authors.initials: R
authors.lastname: Maibach
authors.forename: Jon
authors.initials: J
authors.lastname: Pritchard
authors.forename: Liz
authors.initials: L
authors.lastname: Shafford
authors.forename: Joszef
authors.initials: J
authors.lastname: Zsiros
authors.forename: Piotr
authors.initials: P
authors.lastname: Czaudzerna
authors.forename: Giorgio
authors.initials: G
authors.lastname: Perilongo
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D018197:Hepatoblastoma / Q000097:blood* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000097:blood* / Q000473:pathology / Q000628:therapy; D008297:Male; D016896:Treatment Outcome; D000509:alpha-Fetoproteins / Q000378:metabolism*
chemical_list: D000509:alpha-Fetoproteins