Abstract
Background and Aims: Malignant liver tumors in children are rare and national outcomes for this group are rarely reported. This study mapped the paediatric liver tumors in Denmark from 1985 through 2020 and reports on incidence, outcome and long-term adverse events.
Methods: We identified all paediatric malignant liver tumors from the Danish Childhood Cancer and National Pathology Registries and reviewed the case records for patient and tumor characteristics, treatment and clinical outcome.
Results: We included 79 patients with malignant liver tumors in the analyses; Overall crude incidence was ∼2.29 per 1 million children (<15y) per year, with 61 hepatoblastomas, 9 hepatocellular carcinomas and 9 other hepatic tumors. Overall, 5 y survival was 84%, 78% and 44% respectively. For hepatoblastomas, age > 8 y and diagnosis before year 2000 were statistical significant predictors of a poorer outcome. Liver transplantation was performed in 16% of the cases with an overall survival of 80% compared to 84% for patients receiving surgery. Adverse events were: Reduced renal function in 12% (GFR<90), reduced cardiac function in 7% (EF<55) and impaired hearing function in 48% (Boston 2-4).
Conclusions: Survival for hepatoblastomas in Denmark is increasing and comparable with international results. Stratifying treatment factors in current protocols were confirmed to be significant in the Danish cohort. Hearing loss is the major treatment related side effect and affects about 50% of the patients.
Methods: We identified all paediatric malignant liver tumors from the Danish Childhood Cancer and National Pathology Registries and reviewed the case records for patient and tumor characteristics, treatment and clinical outcome.
Results: We included 79 patients with malignant liver tumors in the analyses; Overall crude incidence was ∼2.29 per 1 million children (<15y) per year, with 61 hepatoblastomas, 9 hepatocellular carcinomas and 9 other hepatic tumors. Overall, 5 y survival was 84%, 78% and 44% respectively. For hepatoblastomas, age > 8 y and diagnosis before year 2000 were statistical significant predictors of a poorer outcome. Liver transplantation was performed in 16% of the cases with an overall survival of 80% compared to 84% for patients receiving surgery. Adverse events were: Reduced renal function in 12% (GFR<90), reduced cardiac function in 7% (EF<55) and impaired hearing function in 48% (Boston 2-4).
Conclusions: Survival for hepatoblastomas in Denmark is increasing and comparable with international results. Stratifying treatment factors in current protocols were confirmed to be significant in the Danish cohort. Hearing loss is the major treatment related side effect and affects about 50% of the patients.
Originalsprog | Engelsk |
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Tidsskrift | Pediatric Blood & Cancer |
Vol/bind | 70 |
Udgave nummer | Supp. 8 |
ISSN | 1545-5009 |
DOI | |
Status | Udgivet - 2. nov. 2024 |
Begivenhed | 55th Congress of the international Society of Pediatric Oncology (SIOP) - Shaw Centre , Ottowa, Canada Varighed: 11. okt. 2023 → 14. okt. 2023 |
Konference
Konference | 55th Congress of the international Society of Pediatric Oncology (SIOP) |
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Lokation | Shaw Centre |
Land/Område | Canada |
By | Ottowa |
Periode | 11/10/2023 → 14/10/2023 |