Hepatoblastoma: 16-years’ experience from a tertiary cancer centre in India

Abstract

BackgroundHepatoblastoma is a rare pediatric tumor arising from the liver. The present study was conducted to ascertain the clinical profile and survival outcomes of patients with hepatoblastoma treated at our centre. MethodsWe collected the case records of patients with hepatoblastoma treated between January 2000 to December 2016 and analysed the baseline characteristics, treatment details and outcomes. Survival was analysed using Kaplan Meier method. ResultsTwenty-seven patients with hepatoblastoma received treatment at our centre during the study period. Median age of the patients was 12 months and 76% were males. The commonest presenting symptom was abdominal mass and the median Alpha Fetoprotein (AFP) level at the time of diagnosis was 40,000 ng/ml. PRETEXT stage II was documented in 11 and III in 11 patients. High risk disease (PRETEXT IV or metastatic disease or portal venous invasion or AFP < 100 ng/ml) was documented in 8/27 (30%) patients. Neoadjuvant chemotherapy (NACT) was given to 23/27 patients and complete surgical resection was possible in 15/23 (65%) after NACT. Infusional cisplatin and doxorubicin (PLADO) was given in 24/27 patients. Liver transplantation was done in 1 patient. The median follow-up was 51 months and the 5-year overall survival for standard risk and high-risk patients was 78.8% and 40% respectively. ConclusionPatients with standard risk hepatoblastoma have survival outcomes comparable to Western countries, however, outcomes in patients with high risk non-metastatic inoperable disease remains low due to financial constraints in performing liver transplantation. Multimodality treatment including NACT with PLADO based regimens followed by resection is a feasible strategy.