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Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience

2008; Elsevier BV; Volume: 44; Issue: 4 Linguagem: Inglês

10.1016/j.ejca.2007.11.022

1879-0852

Maria Antonietta De Ioris, Laurence Brugières, Arthur Zimmermann, Jean W. Keeling, Penelope Brock, Rudolf Maibach, Jon Pritchard, Liz Shafford, Jószef Zsiros, Piotr Czaudzerna, Giorgio Perilongo,

Hepatocellular Carcinoma Treatment and Prognosis

To investigate the characteristics of patients with hepatoblastoma and low serum alpha-fetoprotein (AFP) at diagnosis.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.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.only 9 patients achieved a partial response and 16 died. Median survival: 4.4 months. Two-year overall survival: 24% (confidence interval 10-45%).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.