Multidisciplinary Management of Hepatoblastoma: Three Years of Experience at a Single Centre

Year 2024, Volume: 24 Issue: 2, 70 - 77, 15.08.2024

Esra Arslantaş , Ali Ayçiçek , Sibel Akpınar Tekgündüz , Saide Ertürk , Nazlı Gülsüm Akyel , Banu Yılmaz Özgüven , Erdem Kınacı , İlgin Özden

https://doi.org/10.26650/jchild.2024.1477676

Abstract

Objective: The aim of this study was to report the short-term outcomes of paediatric hepatoblastoma managed by hepatobiliary surgery and paediatric oncology from a single centre.

Methods: Children with hepatoblastoma diagnosed between May 2020 and February 2023 and treated comprehensively in a single centre, were retrospectively reviewed. Management was multidisciplinary and followed the SIOPEL protocols (SIOPEL III-IV).

Results: Eight paediatric patients with a median (range) age at diagnosis of 24 (5-68) months were included. The most common complaint was abdominal distension. There were no patients with PRETEXT stage I. Patients were graded as stage II (n=4), stage III (n=2), and stage IV (n=2). Half of the patients were classified in the standard risk group and the other half in the high-risk group. Chemotherapy was initiated in seven patients, and one was transferred directly to surgery for overt rupture. After neoadjuvant chemotherapy, complete response (CR) was not achieved in any patient, partial response (PR) was achieved in half (n=4), progressive disease and stable disease were present in one patient each, and one patient died. Four patients underwent radical hepatectomy with negative surgical margins after chemotherapy. The median (range) follow-up period was 18 (2-47) months. No recurrence was observed in any patient during follow-up, and the overall and event-free survival rates were 88% and 75%, respectively.

Conclusions: The collective work of surgery and paediatric oncology in management is essential to achieve optimal results.

Keywords

Hepatoblastoma, children, hepatectomy, chemotherapy

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