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

• Published in: European journal of cancer (1990) Vol. 49; no. 12; pp. 2698 - 2704
• Main Authors: Weeda, V.B, Murawski, M, McCabe, A.J, Maibach, R, Brugières, L, Roebuck, D, Fabre, M, Zimmermann, A, Otte, J.B, Sullivan, M, Perilongo, G, Childs, M, Brock, P, Zsíros, J, Plaschkes, J, Czauderna, P, Aronson, D.C
• Format: Journal Article
• Language: English
• Published: Kidlington Elsevier Ltd 01-08-2013; Elsevier
• Subjects: Adolescent; Antineoplastic agents; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Carboplatin - administration & dosage; Carcinoma; Carcinoma, Hepatocellular - drug therapy; Carcinoma, Hepatocellular - surgery; Child; Child, Preschool; Cisplatin - administration & dosage; Clinical oncology; Cohort Studies; Combined Modality Therapy; Doxorubicin - administration & dosage; Female; Fibrolamellar hepatocellular carcinoma; Hematology, Oncology and Palliative Medicine; Hepatectomy - methods; Hepatocellular; Hepatoma; Humans; Infant; Kaplan-Meier Estimate; Liver - drug effects; Liver - pathology; Liver - surgery; Liver diseases; Liver neoplasms; Liver Neoplasms - drug therapy; Liver Neoplasms - surgery; Male; Medical sciences; Multiple tumors. Solid tumors. Tumors in childhood (general aspects); Neoplasms; Paediatrics; Pharmacology. Drug treatments; Treatment Outcome; Tumors; Hepatocellular; Carcinoma; Liver diseases; Fibrolamellar hepatocellular carcinoma; Clinical oncology; Hepatoma; Antineoplastic agents; Paediatrics; Liver neoplasms; Neoplasms; Antineoplastic agent; Genetic variability; Hepatocellular carcinoma; Hepatic disease; Liver cancer; Cancerology; Liver tumor; Child; Human; Paediatrics Liver neoplasms; Genotype; Malignant tumor; Survival; Recommendation; Variant; Treatment; Digestive diseases; Strategy; Cancer
• ISSN: 0959-8049; 1879-0852
• DOI: 10.1016/j.ejca.2013.04.012

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.