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

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Tiusanen , T , Hukkinen , M , Leskinen , O , Soini , T , Kanerva , J A , Jahnukainen , T , Makisalo , H , Heikinheimo , M & Pakarinen , M P 2020 , ' Incidence and long-term outcomes of surgically treated childhood hepatic malignancies in Finland ' , Acta Paediatrica , vol. 109 , no. 2 , pp. 404-414 .

Title: Incidence and long-term outcomes of surgically treated childhood hepatic malignancies in Finland
Author: Tiusanen, Toivo; Hukkinen, Maria; Leskinen, Outi; Soini, Tea; Kanerva, Jukka A.; Jahnukainen, Timo; Makisalo, Heikki; Heikinheimo, Markku; Pakarinen, Mikko P.
Contributor organization: Children's Hospital
HUS Children and Adolescents
Helsinki University Hospital Area
Lastenkirurgian yksikkö
HUS Medical Imaging Center
HUS Abdominal Center
IV kirurgian klinikka
Department of Surgery
Helsinki One Health (HOH)
Date: 2020-02
Language: eng
Number of pages: 11
Belongs to series: Acta Paediatrica
ISSN: 0803-5253
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 1.1:10(6). 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 5-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 = .040), with PRETreatment EXTent of disease IV (PRETEXT, 60% vs 100%, P = .004), and with recurrent disease (67% vs 88%, P = .029). Recurrent/residual disease associated with decreased 5-year survival in hepatocellular carcinoma (0% vs 83%, P = .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.
Subject: children
hepatocellular carcinoma
liver cancer
liver transplantation
3123 Gynaecology and paediatrics
Peer reviewed: Yes
Rights: unspecified
Usage restriction: openAccess
Self-archived version: acceptedVersion

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