Abstract

Objective To explore the treatment and prognosis of advanced stage childhood hepatoblastoma with pulmonary metastasis. Methods Fifty-six cases of advanced stage hepatoblastoma with pulmonary metastasis diagnosed through pathology from April 2006 to June 2014 in Department of Pediatrics, Beijing Tongren Hospital Affiliated to Capital Medical University were enrolled, among them 33 cases were males and 23 cases were females, and the median age was 2.33 years old (1 month-15 years and 1 month old). The clinical effects of multidisciplinary therapy were analyzed. Results (1)Follow-up studies were conducted till December 2016, in which 21 cases of 56 children achieved complete remission, the complete remission rate was 37.5% (21/56 cases), while 12 cases were partial remission and 14 cases were deceased, and the effective rate reached 58.9% (33/56 cases). The follow-up period of 41 children were over 24 months, in which the 2-year free event survival (EFS) rate was 37.5%, 2-year overall survi-val (OS)rate was 75.0%, 5-year survival rate was 42.4%, and the 95% average survival confidence interval was 35.7-55.9 months.(2)The OS rate of children with small age (≤3 years old) was 88.1%(36/42 cases), the ove-rall prognosis was better than that of >3 years old children (35.7%, 5/14 cases) (P=0.003). The survival rate of children with complete tumor resection [OS rate was 89.2%(33/37 cases)] was significantly higher than that of the incomplete excision [OS rate was 47.4%(9/19 cases)], and the difference was statistically significant(P=0.001). The prognosis of epithelial type cases was better than that of other types, and the difference was statistically significant(P<0.05), while the fetal type prognosis was the best, and the difference was statistically significant(χ2=8.56, P=0.014). The growth of alpha fetoprotein was negatively correlated with the clinical efficacy and prognosis(r=-0.468, P=0.023). Conclusions Lung is the most common metastatic site of hepatoblastoma, and the marginal lung metastasis is more common.With insidious onset and poor prognosis.Therefore, it should be treated with early diagnosis and multidisciplinary therapy to improve prognosis. Key words: Hepatoblastoma; Pulmonary metastasis; Chemotherapy; Child; Alpha fetoprotein; Prognosis

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