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Acta Paediatr. 2019 Jul 26. doi: 10.1111/apa.14952. [Epub ahead of print]

Incidence and long-term outcomes of surgically treated childhood hepatic malignancies in Finland.

Author information

1
Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
2
Pediatric Research Center, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
3
Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
4
HUS Medical Imaging Center, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
5
Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
6
Department of Liver and Transplantation Surgery, University Hospital, University of Helsinki, Helsinki, Finland.
7
Department of Pediatrics, Washington University in St. Louis, MO, USA.

Abstract

AIM:

To analyse incidence, treatment, and outcomes of paediatric liver malignancies in Finland during 1987-2017.

METHODS:

Medical records and national cancer registry data of 47 children with liver malignancies were reviewed. Survival was calculated with the Kaplan-Meier method.

RESULTS:

During follow up, liver malignancy incidence remained stable at 1.1:106 . Altogether 42 patients with hepatoblastoma (n=24), hepatocellular carcinoma (n=11), and undifferentiated embryonal sarcoma (n=7) underwent surgery at median age 4.6 (interquartile range, 2.0-9.6) years and were followed up for 13 (7.0-19) years. Cumulative five-year survival was 86% for hepatoblastoma, 41% for hepatocellular carcinoma, and 67% for undifferentiated embryonal sarcoma. Five-year survival was decreased among hepatoblastoma patients aged ≥2.4 years (73% versus 100%, p=0.040), with PRETreatment EXTent of disease IV (PRETEXT, 60% versus 100%, p=0.004), and with recurrent disease (67% versus 88%, p=0.029). Recurrent/residual disease associated with decreased five-year survival in hepatocellular carcinoma (0% versus 83%, p=0.028). Survival was similar among 19 transplanted and 23 resected patients. In total 14 deaths occurred either for the underlying malignancy (n=8), adverse effects of chemotherapy (n=5), or unrelated reasons (n=1).

CONCLUSION:

Outcomes for PRETEXT I-III hepatoblastoma and un-metastasized hepatocellular carcinoma were encouraging. Adverse effects of chemotherapy significantly contributed to mortality. This article is protected by copyright. All rights reserved.

KEYWORDS:

Children; hepatoblastoma; hepatocellular carcinoma; liver cancer; liver transplantation

PMID:
31350767
DOI:
10.1111/apa.14952

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