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Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience
Maretta De Ioris1, Laurence Brugieres, Arthur Zimmermann
1Department of Paediatric Oncology, Institut Gustave Roussy, 39, Rue Camille Desmoulins, 94805 Villejuif, France.
Aim Of The Study:
To investigate the characteristics of patients with hepatoblastoma and low serum alpha-fetoprotein (AFP) at diagnosis.
Patients And 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.
Outcome:
only 9 patients achieved a partial response and 16 died. Median survival: 4.4 months. Two-year overall survival: 24% (confidence interval 10-45%).
Conclusion:
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.
