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. 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

V B Weeda  1 , M Murawski, A J McCabe, R Maibach, L Brugières, D Roebuck, M Fabre, A Zimmermann, J B Otte, M Sullivan, G Perilongo, M Childs, P Brock, J Zsíros, J Plaschkes, P Czauderna, D C Aronson
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Free article

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

V B Weeda et al. Eur J Cancer. 2013 Aug.
Free article

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.

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