Hepatoblastoma associated with trisomy 18

Abstract There is currently no consensus regarding the treatment strategy for malignant tumors with trisomy 18 because of the high mortality rate of this genetic disorder. In this study, we reported five cases of hepatoblastoma associated with trisomy 18. With the exception of one patient with severe cardiac anomalies, the other four patients underwent hepatectomy, and two patients also underwent chemotherapy. There was no treatment-related deterioration of their general condition. Three of the four patients who underwent hepatectomy, including one with a high-risk hepatoblastoma, were still alive at a median follow-up of 43 months (range, 25–169 months) with no sign of recurrence. The combination of radical surgery and chemotherapy is a feasible and effective treatment for hepatoblastoma in carefully selected patients with trisomy 18. Risk adapted management might improve the prognosis and quality of life of these children, even in cases with high-risk hepatoblastoma.