Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation

The most common primary malignant liver tumor of childhood, hepatoblastoma has increased in incidence over the last 30 years, but little is still known about its pathogenesis. Discoveries in molecular biology provide clues but have yet to define targeted therapies. Disease-free survival varies accor...

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Main Authors: Irene Isabel P. Lim, Alexander J. Bondoc, James I. Geller, Gregory M. Tiao
Format: Article
Language:English
Published: MDPI AG 2018-12-01
Series:Children
Subjects:
Online Access:http://www.mdpi.com/2227-9067/6/1/1
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author Irene Isabel P. Lim
Alexander J. Bondoc
James I. Geller
Gregory M. Tiao
author_facet Irene Isabel P. Lim
Alexander J. Bondoc
James I. Geller
Gregory M. Tiao
author_sort Irene Isabel P. Lim
collection DOAJ
description The most common primary malignant liver tumor of childhood, hepatoblastoma has increased in incidence over the last 30 years, but little is still known about its pathogenesis. Discoveries in molecular biology provide clues but have yet to define targeted therapies. Disease-free survival varies according to stage, but is greater than 90% in favorable risk populations, in part due to improvements in chemotherapeutic regimens, surgical resection, and earlier referral to liver transplant centers. This article aims to highlight the principles of disease that guide current treatment algorithms. Surgical treatment, especially orthotopic liver transplantation, will also be emphasized in the context of the current Children’s Oncology Group international study of pediatric liver cancer (AHEP-1531).
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spelling doaj.art-22b131ef6ae74698863d8e8cc20278e22022-12-21T23:24:28ZengMDPI AGChildren2227-90672018-12-0161110.3390/children6010001children6010001Hepatoblastoma—The Evolution of Biology, Surgery, and TransplantationIrene Isabel P. Lim0Alexander J. Bondoc1James I. Geller2Gregory M. Tiao3Division of General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, USADivision of General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, USADivision of Oncology, Cancer and Blood Diseases Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, USADivision of General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, USAThe most common primary malignant liver tumor of childhood, hepatoblastoma has increased in incidence over the last 30 years, but little is still known about its pathogenesis. Discoveries in molecular biology provide clues but have yet to define targeted therapies. Disease-free survival varies according to stage, but is greater than 90% in favorable risk populations, in part due to improvements in chemotherapeutic regimens, surgical resection, and earlier referral to liver transplant centers. This article aims to highlight the principles of disease that guide current treatment algorithms. Surgical treatment, especially orthotopic liver transplantation, will also be emphasized in the context of the current Children’s Oncology Group international study of pediatric liver cancer (AHEP-1531).http://www.mdpi.com/2227-9067/6/1/1hepatoblastomalivercancertransplant
spellingShingle Irene Isabel P. Lim
Alexander J. Bondoc
James I. Geller
Gregory M. Tiao
Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation
Children
hepatoblastoma
liver
cancer
transplant
title Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation
title_full Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation
title_fullStr Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation
title_full_unstemmed Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation
title_short Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation
title_sort hepatoblastoma the evolution of biology surgery and transplantation
topic hepatoblastoma
liver
cancer
transplant
url http://www.mdpi.com/2227-9067/6/1/1
work_keys_str_mv AT ireneisabelplim hepatoblastomatheevolutionofbiologysurgeryandtransplantation
AT alexanderjbondoc hepatoblastomatheevolutionofbiologysurgeryandtransplantation
AT jamesigeller hepatoblastomatheevolutionofbiologysurgeryandtransplantation
AT gregorymtiao hepatoblastomatheevolutionofbiologysurgeryandtransplantation