Abstract

Hepatoblastoma is a rare but the most common solid tumor in children. The incidence is gradually increasing. The international collaboration among four centers in the world has greatly improved the prognosis of hepatoblastoma. They formed the Children’s Hepatic Tumor International Collaboration (CHIC) to standardize the staging system (2017 PRETEXT system) and the risk factors for tumor stratification. Multimodal therapy has become the standard for the management of hepatoblastoma, including surgical resection, liver transplantation, chemotherapy, and so on. Surgery is the primary treatment of early stage hepatoblastoma. Three-dimensional reconstruction is helpful for preoperative evaluation of large tumors, assisting extended hepatectomy for patients in PRETEXT III or IV. Neoadjuvant therapy is useful for reducing the tumor volume and increasing the resectability. Primary liver transplantation is recommended for advanced hepatoblastoma. The lungs are the most common metastatic organ, the treatment of which is critical for the patient’s long-term survival. We reviewed the recent progress in the diagnosis and treatment of hepatoblastoma.

Highlights

  • Hepatoblastoma is the third most commonly diagnosed intra-abdominal solid tumor [1]

  • Neoadjuvant chemotherapy combined with surgical resection of primary and metastatic lesions can achieve a better prognosis for patients with lung metastases

  • Surgical resection is the primary treatment for hepatoblastoma

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Summary

Introduction

Hepatoblastoma is the third most commonly diagnosed intra-abdominal solid tumor [1]. It is the most common primary hepatic malignancy in children [2]. Its absolute incidence is very low, its growth rate is gradually increasing, which increased from 1.89 per 1,000,000 in 2000 to 2.16 per 1,000,000 in 2015, with an annual percentage change of 2.2%. This increase mainly occurs in male children between 2 and 4 years of age, which was found to be an independent predictor for short overall survival [5]. With the development of multimodal treatment and cooperation between international organizations, the prognoses have been greatly improved in recent years [6]

Diagnosis
The children’s hepatic tumor international collaboration (CHIC)
Treatment
Surgical resection for hepatoblastoma
Preoperative or postoperative chemotherapy
Extended hepatectomy or liver transplantation
Does postoperative complication affect prognosis
Liver transplantation in unresectable hepatoblastoma
Treatment after metastasis
Adult hepatoblastoma
Findings
Conclusions
Full Text
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