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

De Ioris, Maretta; Brugieres, Laurence; Zimmermann, Arthur; Keeling, Jean; Brock, Penelope; Maibach, Rudolf; Pritchard, Jon; Shafford, Liz; Zsiros, Joszef; Czaudzerna, Piotr; Perilongo, Giorgio (2008). Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience. European journal of cancer, 44(4), pp. 545-550. Amsterdam: Elsevier 10.1016/j.ejca.2007.11.022

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

Zimmermann, Arthur

ISSN:

0959-8049

ISBN:

18166449

Publisher:

Elsevier

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:54

Last Modified:

17 Mar 2015 21:59

Publisher DOI:

10.1016/j.ejca.2007.11.022

PubMed ID:

18166449

Web of Science ID:

000255073400016

URI:

https://boris.unibe.ch/id/eprint/22832 (FactScience: 37239)

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