Relapses in hepatoblastoma patients: Clinical characteristics and outcome – Experience of the International Childhood Liver Tumour Strategy Group (SIOPEL)

Abstract Purpose To analyse the clinical characteristics and outcome of hepatoblastoma (HB) patients who relapsed after enrolment on SIOPEL studies 1–3. Patients and methods Analysis of clinical data of all 59 patients (pts) registered in SIOPEL 1–3 studies, who relapsed after achieving complete remission (CR). Results The median time from the initial diagnosis to relapse was 12 months (4–115 m). The site of relapse was lung N = 27, liver N = 21, both liver and lung N = 5 and other N = 5 (missing data-MD: 1 patient). All but 9 pts had an alpha-fetoprotein level >10 ng/mL at the time of relapse. Treatment of the relapse included chemotherapy and surgery N = 25, chemotherapy alone N = 21, surgery alone N = 7 and only palliative treatment N = 5 (MD: 1 pt). Overall, 31 pts (52%) achieved a second CR. With a median follow-up of 83 months, 23 pts are alive, (18 in 2nd CR, 5 after a second relapse) and 36 pts have died (35 from disease and 1 from complications). Three-year event-free survival and overall survival are 34% and 43% respectively (95% confidence interval [CI] 0.28–0.69). The main factors associated with a good outcome were PRETEXT group I–III at diagnosis, a high AFP level at relapse and relapse treatment including both chemotherapy and surgery. Conclusion Relapses in HB are rare events occurring in less than 12% of pts after CR. Combined treatment with chemotherapy and surgical removal of the tumour is essential for long-term survival.