Adult hepatoblastoma: a review of 47 cases.

Background: Adult hepatoblastoma is a very rare and aggressive neoplasm. The aim of this study was to review the cases of adult hepatoblastoma patients.

Methods: A search of all case reports about adult hepatoblastoma from 1958 to 2016 by medical database was performed. Data about demographic information, clinical symptoms and signs, laboratory test and imaging examination, treatment and clinical outcomes were extracted and analysed.

Results: A total of 43 English articles including 47 cases of adult hepatoblastoma patients were collected. The median age was 39 years (range: 18-84 years), including 25 males and 22 females. Hepatitis B virus tests were positive in seven patients. Alpha-fetoprotein was positive in 18 patients. Liver cirrhosis was confirmed in six cases. The median size of main tumour nodule was 15 cm (range: 6-42 cm) in 30 patients with details. Hepatectomy was undertaken in 28 cases, exploratory laparotomy in seven cases and chemoembolization and chemotherapy in two cases. Follow-up data were available in 33 patients including 23 patients with liver resection and the median survival time was 8 months (range: 0-151 months) and the 1-year survival rate was 39.2%. For 23 patients with liver resection, the median survival time was 15 months (range: 0.5-151 months) and the 1-year survival rate was 54.2%. In Cox multivariate analysis, liver resection was indentified as an independent prognostic factor for prolonged survival (P = 0.001).

Conclusion: Curative liver resection can prolong survival of adult hepatoblastoma. More effective systemic therapies may need to provide a great survival benefit.