Book ChapterDOI
Pediatric Hepatoblastoma. Complete Surgery and Ultrasound Monitoring
Daniela Iacob,Otilia Fufezan,Alexandru Serban +2 more
- pp 263-274
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TLDR
Referral to a transplant center during the first cycles of chemotherapy appears to offer the best opportunity for long-term survival.Abstract:
Hepatoblastoma is the most common primary liver tumor of childhood. Most hepatoblastoma cases are sporadic, but others are associated with inherited conditions, suggesting the importance of genetic abnormalities in the pathogenesis and progression of this disease. A diverse range of cytogenetic alterations have been reported, as significant gains of genetic material, with various differences in the number and type of alterations between the different histologic components of hepatoblastoma. They could represent diagnostic marker, they may play a role in the tumorigenesis of hepatoblastoma and might become potential target for molecular therapeutics. An elevated serum alpha-fetoprotein is useful both at diagnosis of hepatoblastoma and in disease monitoring, by determining the success of surgical resection and/or response to adjuvant chemotherapy. Imaging has a significant role in the evaluation of hepatoblastoma, offering data about localization, resectability, and follow-up. Ultrasonography is the best initial imaging investigation of a child with a liver mass, allowing real-time investigation of the tumour. Complete surgical resection together with pre- and postoperative chemotherapy are the mainstay of treatment of hepatoblastoma. The potential down-staging effect of neoadjuvant chemotherapy on hepatoblastoma might facilitate remission and convert unresectable tumors into operable ones. Radical resection can be obtained either conventionally by partial hepatectomy or with orthotopic liver transplant. Complete hepatectomy with living donor liver transplantation provides optimal surgical treatment in unresectable stage III and initial stage IV disease confined to the liver at resection. Referral to a transplant center during the first cycles of chemotherapy appears to offer the best opportunity for long-term survival.read more
References
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Journal ArticleDOI
Convergence of Wnt, ß-Catenin, and Cadherin Pathways
W. James Nelson,Roel Nusse +1 more
TL;DR: Evidence is assembled of possible interrelations between Wnt and other growth factor signaling, β-catenin functions, and cadherin-mediated adhesion in tissue differentiation.
Journal ArticleDOI
Risk-adapted treatment for childhood hepatoblastoma. final report of the second study of the International Society of Paediatric Oncology--SIOPEL 2.
Giorgio Perilongo,E. Shafford,Rudolf Maibach,Doron Aronson,Laurence Brugières,Penelope Brock,Margaret Childs,Piotr Czauderna,G. MacKinlay,J.B. Otte,Jon Pritchard,R. Rondelli,Marcelo Scopinaro,C.R. Staalman,Jack Plaschkes +14 more
TL;DR: A treatment strategy based on CDDP monotherapy and surgery thus appears effective in SR-HB but, despite CT intensification, only half of the HR-HB patients are long-term survivors.
Journal ArticleDOI
Guidelines for surgical treatment of hepatoblastoma in the modern era - Recommendations from the childhood liver tumour strategy group of the international society of paediatric oncology (SIOPEL)
Piotr Czauderna,Jean-Bernard Otte,Daniel C. Aronson,Frédéric Gauthier,Gordon A. MacKinlay,Derek J. Roebuck,J. Plaschkes,Giorgio Perilongo +7 more
TL;DR: The surgical recommendations of the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology are presented to stimulate international debate on this issue and recommend the more frequent use of orthotopic liver transplant, as well as the standardisation of techniques for partial liver resection.
Journal ArticleDOI
Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience
Maretta De Ioris,Laurence Brugières,Arthur Zimmermann,Jean W. Keeling,Penelope Brock,Rudolf Maibach,Jon Pritchard,Liz Shafford,Joszef Zsiros,Piotr Czaudzerna,Giorgio Perilongo +10 more
TL;DR: This study clearly identifies patients with hepatoblastoma and low serum AFP at diagnosis as a high-risk subgroup with extensive disease at diagnosis, poor response to chemotherapy and a poor outcome.
Journal ArticleDOI
The emerging family of hepatoblastoma tumours: from ontogenesis to oncogenesis.
TL;DR: An impressive amount of molecular biological information on liver ontogenesis and growth regulation of hepatic tumours has recently accumulated, which will allow the development of a comprehensive classification system with particular emphasis on prognostics.