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Eur J Cancer. 2008 Mar;44(4):545-50. doi: 10.1016/j.ejca.2007.11.022. Epub 2007 Dec 31.

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

Author information

1
Department of Paediatric Oncology, Institut Gustave Roussy, 39, Rue Camille Desmoulins, 94805 Villejuif, France.

Abstract

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.

PMID:
18166449
DOI:
10.1016/j.ejca.2007.11.022
[Indexed for MEDLINE]

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