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Review
. 2018 Dec 21;6(1):1.
doi: 10.3390/children6010001.

Hepatoblastoma-The Evolution of Biology, Surgery, and Transplantation

Irene Isabel P Lim  1 , Alexander J Bondoc  2 , James I Geller  3 , Gregory M Tiao  4
Affiliations
Review

Hepatoblastoma-The Evolution of Biology, Surgery, and Transplantation

Irene Isabel P Lim et al. Children (Basel). .

Abstract

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).

Keywords: cancer; hepatoblastoma; liver; transplant.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Liver sections [4]. The liver has four sections, separated by the hepatic and portal veins: (a) right posterior, (b) right anterior, (c) left medial, and (d) left lateral. Note that the caudate lobe is not considered part of these sections.
Figure 2
Figure 2
These stratification trees are used as the framework for management of hepatoblastoma in the AHEP-1531 study [5]. AFP = alpha-fetoprotein.
Figure 3
Figure 3
Treatment strategy under AHEP-1531. Management varies based on risk (very low to high) and PRETEXT stage. Cis = cisplatin; C5VD = cisplatin, 5-fluorouracil, vincristine, doxorubicin; CD = carboplatin, doxorubicin; VI/CE = vincristine, irinotecan, or carboplatin, etoposide; WDF = well-differentiated fetal; SIOPEL = Société Internationale d’Oncologie Pédiatrique.
Figure 4
Figure 4
Indocyanine green (ICG) aids in localization of pulmonary metastases. The lesion is indistinguishable from normal lung parenchyma (A), but ICG fluorescence clearly delineates the metastatic lesion (B).
See this image and copyright information in PMC

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