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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

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

European journal of cancer (1990), 2013-08, Vol.49 (12), p.2698-2704 [Periódico revisado por pares]

Kidlington: Elsevier Ltd

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Título:
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
Autor: 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
Assuntos: 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
É parte de: European journal of cancer (1990), 2013-08, Vol.49 (12), p.2698-2704
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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Descrição: 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.
Editor: Kidlington: Elsevier Ltd
Idioma: Inglês

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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

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

Kidlington: Elsevier Ltd

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