The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials
TLDR
Successive SIOPEL trials and increasing international collaboration have improved survival rates of patients with HB through risk stratification, advances in chemotherapy and increased complete resection rates including liver transplantation as a surgical option.Abstract:
Aim of the Review: To describe the significant improvement in the diagnosis, treatment and outcome of children diagnosed with hepatoblastoma (HB) that has occurred in the past four decades. Recent findings are mainly focused on lessons learned from the experiences of the Childhood Liver Tumors Strategy Group (SIOPEL). Important milestones were the risk stratification of HB that allowed to tailor down therapy for standard-risk HB and intensify treatment for high-risk HB. The multi-institutional international cooperative SIOPEL trials are reviewed and current treatment guidelines are given. Intensified cooperation between the SIOPEL and the Children's Oncology Group (COG) and the national study groups from Germany (GPOH) and Japan (JPLT) led to the acceptance and use of one staging system (PRETEXT) and the formation of a single robust database containing data of 1605 HB patients. This will allow analysis with enough statistical power of treatment directing factors that will form one of the bases of the next-generation clinical trial that is currently designed by all four collaborating study groups. Summary: Successive SIOPEL trials and increasing international collaboration have improved survival rates of patients with HB through risk stratification, advances in chemotherapy and increased complete resection rates including liver transplantation as a surgical option.read more
Citations
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Journal ArticleDOI
Malignant tumors of the liver in children.
TL;DR: An overview of pediatric liver tumors; in particular of the two most frequently occurring groups of hepatoblastomas and hepatocellular carcinomas is given.
Journal ArticleDOI
New insights into diagnosis and therapeutic options for proliferative hepatoblastoma
Katarzyna B. Hooks,Jérôme Audoux,Helena Fazli,Sarah Lesjean,Tony Ernault,Tony Ernault,Nathalie Dugot-Senant,Thierry Leste-Lasserre,Martin Hagedorn,Benoit Rousseau,Coralie Danet,Sophie Branchereau,Laurence Brugières,Sophie Taque,Catherine Guettier,Monique Fabre,Anne Rullier,Marie-Annick Buendia,Marie-Annick Buendia,Thérèse Commes,Christophe Grosset,Anne-Aurélie Raymond +21 more
TL;DR: The highly proliferating C2A subtype of hepatoblastoma is characterized by topoisomerase 2‐alpha gene up‐regulation and FA pathway activation, and the HB therapeutic arsenal could include bortezomib for the treatment of patients with the most aggressive tumors.
Journal ArticleDOI
Epigenetic footprint enables molecular risk stratification of hepatoblastoma with clinical implications
Juan Carrillo-Reixach,Laura Torrens,Laura Torrens,Marina Simon-Coma,Laura Royo,Montserrat Domingo-Sàbat,Jordi Abril-Fornaguera,Jordi Abril-Fornaguera,Nicholas K. Akers,Nicholas K. Akers,Margarita Sala,Sonia Ragull,Magdalena Arnal,Núria Villalmanzo,Stefano Cairo,Alberto Villanueva,Roland Kappler,Marta Garrido,Laura Guerra,Constantino Sábado,Gabriela Guillén,Mar Mallo,David Piñeyro,Maria Vázquez-Vitali,Olga Kuchuk,María Elena Mateos,Gema Ramírez,Manuel López Santamaría,Yasmina Mozo,Aroa Soriano,Michael A. Grotzer,Sophie Branchereau,Nagore García de Andoin,Blanca López-Ibor,Ricardo López-Almaraz,José Antonio Salinas,Bárbara Torres,Francisco Andrés Pérez Hernández,José Javier Uriz,Monique Fabre,Julià Blanco,Claudia Paris,Viera Bajčiová,Genevieve Laureys,Helena Masnou,Ariadna Clos,Cristina Beléndez,Catherine Guettier,Lauro Sumoy,Ramon Planas,Mireia Jordà,Lara Nonell,Piotr Czauderna,Bruce Morland,Daniela Sia,Bojan Losic,Marie Annick Buendia,Maria Rosa Sarrias,Josep M. Llovet,Josep M. Llovet,Josep M. Llovet,Carolina Armengol +61 more
TL;DR: The first Molecular Risk Stratification of HB (MRS-HB), which encompasses three main prognostic categories and improves the current clinical risk stratification approach, is defined and expands knowledge about the molecular features of HB.
Journal ArticleDOI
DPEP1 is a direct target of miR-193a-5p and promotes hepatoblastoma progression by PI3K/Akt/mTOR pathway.
Xichun Cui,Xin Liu,Qicai Han,Jianming Zhu,Jianhao Li,Zhigang Ren,Liwen Liu,Yanbing Luo,Zhifang Wang,Dandan Zhang,Yingzhong Fan,Da Zhang,Gang Dong +12 more
TL;DR: It is shown that DPEP1 was significantly upregulated and was associated with poor prognosis in HB patients and the miR-193a-5p /DPEP1 axis might be a good prognostic predictor and therapeutic target in HB.
Journal ArticleDOI
Hepatoblastoma: current understanding, recent advances, and controversies.
Piotr Czauderna,Hanna Garnier +1 more
TL;DR: The treatment of HB started from one and the same therapy for all patients and aimed at increased treatment individualization, but the future seems to lie in biology-driven patient-tailored therapies.
References
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Journal ArticleDOI
Hepatoblastoma and hepatocarcinoma in infancy and childhood. Report of 47 cases.
Kamal G. Ishak,Paul R. Glunz +1 more
TL;DR: It is considered that the only hope of cure in hepatoblastoma is surgical excision, and early exploration, biopsy, and surgical resection are recommended in all patients.
Journal ArticleDOI
Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of pediatrics surgical section survey — 1974☆
Philip R. Exelby,Philip R. Exelby,Philip R. Exelby,Robert M. Filler,Robert M. Filler,Robert M. Filler,Jay L. Grosfeld,Jay L. Grosfeld,Jay L. Grosfeld +8 more
TL;DR: It seems that operative excision offers the only chance of cure in children with these tumors and cure rates of 60% can be expected with hepatoblastoma and 33% in hepatocellular carcinoma if the tumor can be completely excised.
Journal ArticleDOI
2005 PRETEXT: a revised staging system for primary malignant liver tumours of childhood developed by the SIOPEL group
Derek J. Roebuck,Daniel C. Aronson,Philippe Clapuyt,Piotr Czauderna,Jean de Ville de Goyet,Frédéric Gauthier,Gordon A. MacKinlay,Rudolf Maibach,Kieran McHugh,Øystein E. Olsen,Jean-Bernard Otte,Danièle Pariente,Jack Plaschkes,Margaret Childs,Giorgio Perilongo +14 more
TL;DR: This paper, written by members of the radiology and surgery committees of the International Childhood Liver Tumor Strategy Group (SIOPEL), presents various clarifications and revisions to the original PRETEXT system.
Journal ArticleDOI
Successful Treatment of Childhood High-Risk Hepatoblastoma With Dose-Intensive Multiagent Chemotherapy and Surgery: Final Results of the SIOPEL-3HR Study
Jozsef Zsiros,Rudolf Maibach,E. Shafford,Laurence Brugières,Penelope Brock,Piotr Czauderna,Derek J. Roebuck,Margaret Childs,Arthur Zimmermann,Véronique Laithier,Jean-Bernard Otte,Beatriz de Camargo,Gordon A. MacKinlay,Marcelo Scopinaro,Daniel C. Aronson,Jack Plaschkes,Giorgio Perilongo +16 more
TL;DR: The applied treatment rendered a great proportion of tumors resectable, and, in comparison with previously published results, led to an improved survival in patients with high-risk hepatoblastoma.
Journal ArticleDOI
Pretreatment prognostic factors for children with hepatoblastoma-- results from the International Society of Paediatric Oncology (SIOP) study SIOPEL 1.
Julia Brown,Giorgio Perilongo,E. Shafford,Jean W. Keeling,Jon Pritchard,Penelope Brock,Claire Dicks-Mireaux,Angela Phillips,Anton Vos,Jack Plaschkes +9 more
TL;DR: Tumour focality and enlargement of hilar lymph nodes at diagnosis were univariately associated with EFS, and in multivariate analysis, PRETEXT was the only predictor of OS; PRETEXT and metastases were predictors of EFS.
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