Background: Approximately 20% of patients who have hepatoblastoma (HB) still have unresectable disease after preoperative chemotherapy (POC). In these circumstances, orthotopic liver transplantation (OLT) should be performed 1 month after POC. The authors sought to identify presenting features that would predict unresectability in patients with HB and to provide suggestions for early referral and listing for OLT.
Methods: Notes, radiology films, and histology from patients who were treated over the previous 20 years were reviewed. Unfeasible resection was defined by bilobar involvement, vascular extension, and metastatic disease after POC. Failed conservative treatment (FCT) was used to categorize patients who were not disease-free with their native liver > or =1 year after surgery.
Results: Of 28 patients who were studied, 14 patients underwent resection, and 10 patients required OLT. Four patients did not undergo any type of surgery because of tumor progression. Overall, the 5-year survival rate was 76% (95% confidence interval, 54.8-89%). Predictors of FCT were multifocality (P = .006), a high pretreatment extent of tumor (PRETEXT) score (III or IV; P = .006), portal vein involvement (P = .02), hepatic vein involvement (P = .02), or vena cava involvement (P = .05). Patients who achieved a curative resection presented at a younger age (median, 0.7 years vs 4.2 years, P = .02). Patients who had multifocal lesions and those who had an alpha-fetoprotein (alphaFP) level <100 ng/mL survived only if they underwent transplantation.
Conclusions: Patients with HB who were managed by combined chemotherapy and surgery has a high survival rate. Older patients who had multifocal tumors, high PRETEXT scores, involvement of major liver vessels, and alphaFP levels <100 ng/mL were less likely to achieve curative resection. These findings at presentation should lead the clinicians to liaise early with a transplantation center.