Abstract

Hepatoblastoma is the most common childhood liver malignancy. The management of hepatoblastoma requires multidisciplinary efforts. The five-year overall survival is approximately 80% in developed countries. Surgery remains the mainstay of treatment for hepatoblastoma, and meticulous techniques must be employed to ensure safe and effective local control surgeries. Additionally, there have been several advances from both pediatric and adult literature in the way liver tumor surgery is performed. In this review, we highlight important aspects of liver surgery for hepatoblastoma, the management of metastatic disease, and the most current technical advances in performing these procedures in a safe and effective manner.

Highlights

  • Hepatoblastoma is the most common liver malignancy in children

  • We mainly focus on the surgical resection of hepatoblastoma

  • The pre-treatment extent of tumor (PRETEXT) system was developed by SIOPEL to standardize imaging evaluation and risk stratification for hepatoblastoma prior to neoadjuvant chemotherapy [13,22,23,24], whereas the POST-TEXT system uses the same standards as PRETEXT (Table 1) but classifies hepatoblastoma during neoadjuvant chemotherapy [13,23]

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Summary

Introduction

Hepatoblastoma is the most common liver malignancy in children. Hepatoblastoma accounts for over 90% of the primary hepatic malignancies among children less than 5 years of age [1]. The incidence of hepatoblastoma has increased over the past two decades, partially due to the increased survival of premature and low-birth-weight infants [2,3,4,5]. The five-year overall survival rate for hepatoblastoma is approximately 80% with those who underwent partial hepatectomy achieving survival rates as high as 91% [9]. 85% of the patients with primary liver transplantation [10] These achievements in hepatoblastoma treatment are the results of joint international efforts which have led to the development of treatment guidelines. We mainly focus on the surgical resection of hepatoblastoma

Preoperative Planning
The PRETEXT and POST-TEXT System
Upfront Versus Delayed Surgery
Advancement in Techniques to Make Tumors Resectable
Advances Intraoperative Techniques and Approaches to Local Control
Resection of Lung Metastasis
Management of Disease Relapse
Findings
Conclusions
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