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.
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.
Authors: Elana P Simon; Catherine A Freije; Benjamin A Farber; Gadi Lalazar; David G Darcy; Joshua N Honeyman; Rachel Chiaroni-Clarke; Brian D Dill; Henrik Molina; Umesh K Bhanot; Michael P La Quaglia; Brad R Rosenberg; Sanford M Simon Journal: Proc Natl Acad Sci U S A Date: 2015-10-21 Impact factor: 11.205
Authors: Alexander J Towbin; Rebecka L Meyers; Helen Woodley; Osamu Miyazaki; Christopher B Weldon; Bruce Morland; Eiso Hiyama; Piotr Czauderna; Derek J Roebuck; Greg M Tiao Journal: Pediatr Radiol Date: 2018-02-09
Authors: Kimberly J Riehle; Matthew M Yeh; Jeannette J Yu; Heidi L Kenerson; William P Harris; James O Park; Raymond S Yeung Journal: Mod Pathol Date: 2014-06-13 Impact factor: 7.842
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Authors: David G Darcy; Marcus M Malek; Rachel Kobos; David S Klimstra; Ronald DeMatteo; Michael P La Quaglia Journal: J Pediatr Surg Date: 2014-12-10 Impact factor: 2.545
Authors: Joshua N Honeyman; Elana P Simon; Nicolas Robine; Rachel Chiaroni-Clarke; David G Darcy; Irene Isabel P Lim; Caroline E Gleason; Jennifer M Murphy; Brad R Rosenberg; Lydia Teegan; Constantin N Takacs; Sergio Botero; Rachel Belote; Soren Germer; Anne-Katrin Emde; Vladimir Vacic; Umesh Bhanot; Michael P LaQuaglia; Sanford M Simon Journal: Science Date: 2014-02-28 Impact factor: 47.728
Authors: Roberta Angelico; Chiara Grimaldi; Maria Cristina Saffioti; Aurora Castellano; Marco Spada Journal: Transl Gastroenterol Hepatol Date: 2018-09-10