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Eur J Cancer. 2013 Aug;49(12):2698-704. doi: 10.1016/j.ejca.2013.04.012. Epub 2013 May 15.

Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma--results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience.

Author information

1
Department of Paediatric Surgery, Emma Children's Hospital, AMC, Amsterdam, The Netherlands. v.b.weeda@gmail.com

Abstract

PURPOSE:

Fibrolamellar hepatocellular carcinoma (FL-HCC) and conventional hepatocellular carcinoma (HCC) cases in two consecutive paediatric HCC trials were analysed to compare outcome and derive treatment implications.

PATIENTS AND METHODS:

Data of 24 FL-HCC (24% PRETEXT IV) and 38 HCC (42% PRETEXT IV) cases from SIOPEL-2 and -3 (1995-1998, 1998-2006) were analysed. Patients were treated according to SIOPEL-2 and -3 high-risk protocol (carboplatin+doxorubicin alternating with cisplatin; seven preoperative, three postoperative cycles) or with primary surgery followed by chemotherapy as indicated.

RESULTS:

Thirteen of 24 FL-HCC (54%) and 32/38 HCC (84%) were initially treated with chemotherapy. Eight FL-HCC (33%) and five HCC patients (13%) had primary surgery. Partial response was observed in 31% of FL-HCC versus 53% of HCC patients (p=0.17). Complete resection was achieved in ten FL-HCC and seven HCC patients (p=0.08). Three-year event free survival (EFS) was 22% for FL-HCC versus 28% for HCC. Overall survival (OS) was not significantly different at 3 years follow up (42% for FL-HCC versus 33% for HCC, p=0.24). EFS/OS Kaplan-Meier curves did not differ significantly, with median follow up of 43 (FL-HCC) and 60 (HCC) months. No significant correlation was found between potential prognostic factors and OS. In the entire cohort nine out of 23 (39%) patients with complete resection or orthotopic liver transplantation versus 34/39 (87%) without successful surgical treatment, died.

CONCLUSIONS:

Long-term OS in FL-HCC and HCC is similar. With low response rates, complete resection remains the treatment of choice.

TRIAL REGISTRATION:

ClinicalTrials.gov NCT00003912 NCT00077389.

KEYWORDS:

Antineoplastic agents; Carcinoma; Clinical oncology; Fibrolamellar hepatocellular carcinoma; Hepatocellular; Hepatoma; Liver diseases; Liver neoplasms; Neoplasms; Paediatrics

PMID:
23683550
DOI:
10.1016/j.ejca.2013.04.012
[Indexed for MEDLINE]
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