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

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http://hdl.handle.net/10138/319415

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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 . https://doi.org/10.1111/apa.14952

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: University of Helsinki, Children's Hospital
University of Helsinki, HUS Children and Adolescents
University of Helsinki, HUS Medical Imaging Center
University of Helsinki, HUS Children and Adolescents
University of Helsinki, HUS Children and Adolescents
University of Helsinki, HUS Children and Adolescents
University of Helsinki, HUS Abdominal Center
University of Helsinki, Helsinki One Health (HOH)
University of Helsinki, HUS Children and Adolescents
Date: 2020-02
Language: eng
Number of pages: 11
Belongs to series: Acta Paediatrica
ISSN: 0803-5253
URI: http://hdl.handle.net/10138/319415
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
hepatoblastoma
hepatocellular carcinoma
liver cancer
liver transplantation
LIVER-TRANSPLANTATION
CHILDREN
HEPATOBLASTOMA
CANCER
TUMORS
SURVIVAL
SYSTEM
3123 Gynaecology and paediatrics
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