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Incidence and long‐term outcomes of surgically treated childhood hepatic malignancies in Finland
Author(s) -
Tiusanen Toivo,
Hukkinen Maria,
Leskinen Outi,
Soini Tea,
Kanerva Jukka A.,
Jahnukainen Timo,
Mäkisalo Heikki,
Heikinheimo Markku,
Pakarinen Mikko P.
Publication year - 2020
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/apa.14952
Subject(s) - hepatoblastoma , medicine , hepatocellular carcinoma , malignancy , interquartile range , incidence (geometry) , gastroenterology , cumulative incidence , liver disease , chemotherapy , surgery , transplantation , physics , optics
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 PRET reatment EXT ent 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.