Abstract

BackgroundTo report the outcomes of hepatoblastoma resected in our institution.MethodsWe diagnosed 135 children with hepatoblastoma at our institution between January 2010 and December 2017. Patients who underwent liver resection were included for analysis. However, patients who abandoned treatment after diagnosis were excluded from analysis, but their clinical characteristics were provided in the supplementary material.ResultsForty-two patients abandoned treatment, whereas 93 patients underwent liver resection and were included for statistical analysis. Thirty-six, 23, 3, and 31 patients had PRETEXT stages II, III, IV, and unspecified tumours, respectively. Seven patients had ruptured tumour; 9 had lung metastasis (one patient had portal vein thrombosis concurrently). Sixteen patients underwent primary liver resection; 22, 25, and 30 patients received cisplatin-based neoadjuvant chemotherapy and delayed surgery, preoperative transarterial chemoembolization (TACE) and delayed surgery, and a combination of cisplatin-based neoadjuvant chemotherapy, TACE, and delayed surgery, respectively. Forty patients had both PRETEXT and POST-TEXT information available for analysis. Twelve patients were down-staged after preoperative treatment, including 2, 8, and 2 patients from stages IV to III, III to II, and II to I, respectively. Ten patients with unspecified PRETEXT stage were confirmed to have POST-TEXT stages II (n = 8) and I (n = 2) tumours. Seven tumours were associated with positive surgical margins, and 12 patients had microvascular involvement. During a median follow-up period of 30.5 months, 84 patients survived without relapse, 9 experienced tumour recurrence, and 4 died. The 2-year event-free survival (EFS) and overall survival (OS) rates were 89.4 ± 3.4%, and 95.2 ± 2.4%, respectively; they were significantly better among patients without metastasis (no metastasis vs metastasis: EFS, 93.5 ± 3.7% vs 46.7 ± 19.0%, adjusted p = 0.002. OS, 97.6 ± 2.4% vs 61.0 ± 18.1%, adjusted p = 0.005), and similar among patients treated with different preoperative strategies (chemotherapy only vs TACE only vs Both: EFS, 94.7 ± 5.1% vs 91.7 ± 5.6% vs 85.6 ± 6.7%, p = 0.542. OS, 94.1 ± 5.7% vs 95.7 ± 4.3% vs 96.7 ± 3.3%, p = 0.845).ConclusionThe OS for patients with hepatoblastoma who underwent liver resection was satisfactory. Neoadjuvant chemotherapy and TACE seemed to have a similar effect on OS. However, the abandonment of treatment by patients with hepatoblastoma was common, and may have biased our results.

Highlights

  • To report the outcomes of hepatoblastoma resected in our institution

  • The diagnosis of hepatoblastoma was initially made based on an elevated A combination of elevated α-fetoprotein protein (AFP) level and radiographic detection of a liver mass, and confirmed via pathological examination of samples obtained via either biopsy or primary liver resection

  • The primary outcome was to evaluate the event-free survival and overall survival of hepatoblastoma resected in our institution

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Summary

Introduction

To report the outcomes of hepatoblastoma resected in our institution. Hepatoblastoma is tshe most common childhood liver malignancy, and has a prevalence of 1 per 1,000,000 population [1, 2]. The incidence of hepatoblastoma has increased in the past two decades, and this upward trend has been correlated with an increasing survival rate among premature and low-birth-weight infants [3]. Hepatoblastoma usually affects children younger than 3 years, and presents as a large abdominal mass. Some patients may present with sudden abdominal pain and haemorrhagic shock in the scenario of tumour rupture. A combination of elevated α-fetoprotein protein (AFP) level and radiographically identified hepatic mass suffices for the clinical diagnosis of hepatoblastoma in children with ages between 6 months and 3 years. Biopsy, preferably via ultrasound-guided core needle biopsy is recommended for patients of all age groups [4, 5]

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