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. 2022 Dec;33(12):1069-1078.
doi: 10.5152/tjg.2022.21827.

Multidisciplinary Management of Pediatric Hepatoblastoma: A 20-Year Single-Center Experience

Funda Tayfun Küpesiz  1 , Ayşe Nur Akınel  1 , Hilal Akbaş  2 , Çiğdem Sivrice  1 , Gülen Tüysüz Kintrup  1 , Güngör Karagüzel  3 , Mustafa Melikoğlu  3 , Mustafa Tekinalp Gelen  4 , Bülent Aydınlı  5 , Alphan Küpesiz  1 , Elif Güler  1
Affiliations

Multidisciplinary Management of Pediatric Hepatoblastoma: A 20-Year Single-Center Experience

Funda Tayfun Küpesiz et al. Turk J Gastroenterol. 2022 Dec.

Abstract

Background: Hepatoblastoma is rare cancer that responds well to risk-based chemotherapy, and surgical treatment is needed to achieve complete remission and satisfactory survival rates in hepatoblastoma patients. In this study, we evaluated the clinical features and treatment outcomes of pediatric hepatoblastoma patients treated in our clinic.

Methods: Eighteen patients with hepatoblastoma who were treated and followed up in our center between June 1999 and June 2020 were analyzed retrospectively. All patients were evaluated by a multidisciplinary team and managed using a risk-based protocol (SIOPEL-1 and SIOPEL-3).

Results: The patients' mean age at diagnosis was 38.33 ± 52.34 months. Sixteen patients (89%) received neoadjuvant chemotherapy, and 2 patients (11%) who underwent complete mass excision at diagnosis received adjuvant chemotherapy. After neoadjuvant therapy, the tumor was completely resected in 8 patients (45%), while liver transplantation was performed in 6 patients (34%) because complete resection of the tumor was not possible. Two patients died before surgical treatment. One patient relapsed with lung metastasis after salvage chemotherapy. She is alive without disease at 64 months. The mean follow-up time was 59.3 ± 49.8 months; 5-year overall and disease-free survival rates were 88.9% and 80.8%, respectively. The 5-year overall survival rate was 100% for both liver transplant and resected patients, whereas 5-year disease-free survival was lower in transplant patients (75% vs 100%, P < .001).

Conclusion: Multidisciplinary follow-up is especially important for patients who may need liver transplantation. Some patients may benefit from new treatment options such as radiofrequency ablation and cyberknife treatment.

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Figures

Figure 1.
Figure 1.
Flowchart of the treatments and outcomes of patients with hepatoblastoma. *One patient died without receiving chemotherapy or surgery due to tumor rupture at the start of the first course of chemotherapy.
Figure 2.
Figure 2.
The 5-year overall rates and event-free rates.
Figure 3.
Figure 3.
The 5-year disease-free survival curves of patients with liver transplantation versus hepatic resection.
See this image and copyright information in PMC

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