Therapeutic experience with hepatoblastoma associated with trisomy 18

Abstract

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease-free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.

Surgical and anesthetic management for hepatectomy in two pediatric patients with trisomy 18, pulmonary hypertension, and hepatoblastoma

“…Including the current reports, 46 patients with trisomy 18 and HB have been reported in the literature, including 23 patients reported in the Japanese literature and summarized in English elsewhere (Supporting Information Table S1). These patients were predominantly female (M:F 1:7.8), in contrast to a male predominance for patients not selected for trisomy 18 (M:F 1.2:1) . This appears to be related to the female predominance of patients with trisomy 18 surviving past the neonatal period with the recent increases in life‐sustaining treatment approaches .…”

“…1,2 Survival in infants with trisomy 18 has also improved. 3 There is a well-recognized association between HB and trisomy 18, 4 and as a result, resection of HB in children with trisomy 18 needs to be considered. Repair of congenital heart defects early in life reduces in-hospital mortality in these infants, 5 but some of them have chronic issues with Abbreviations: AFP, alpha-fetoprotein; CVP, central venous pressure; HB, hepatoblastoma; IVC, inferior vena cava; VSD, ventricular septal defect pulmonary hypertension.…”

Interdisciplinary care of children with trisomy 13 and 18

“…Treatment for hepatoblastoma and Wilms' tumor may include surgical resection with or without chemotherapy. Cancer treatment is possible and has been successful in some circumstances but must be balanced with the burden to the child (Inoue et al, 2018). Due to the impact of trisomy 13 and 18 on multiple organ systems, treatment options should be considered collaboratively with an interdisciplinary care team and family.…”

The Genetic Changes of Hepatoblastoma

“…Trisomy 18, regarded as a fatal disease, affects approximately 1 per 6,000 newborns (30). The correlation between trisomy 18 and hepatoblastoma has been reported in several previous studies (21,(31)(32)(33). Tomlinson et al raised an interesting point that the hepatoblastoma cases with trisomy 18 almost were females, and this situation was contrary to hepatoblastoma with higher prevalence in males (34).…”