Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience.
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.
De Ioris
Maretta
M
Department of Paediatric Oncology, Institut Gustave Roussy, 39, Rue Camille Desmoulins, 94805 Villejuif, France.
Brugieres
Laurence
L
Zimmermann
Arthur
A
Keeling
Jean
J
Brock
Penelope
P
Maibach
Rudolf
R
Pritchard
Jon
J
Shafford
Liz
L
Zsiros
Joszef
J
Czaudzerna
Piotr
P
Perilongo
Giorgio
G
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*
D000509:alpha-Fetoproteins