Treatment outcomes of Hepatoblastoma in a resource challenged setting

Abstract

Background and Aim: Hepatoblastoma is the most common malignant tumour in the paediatric age group. Successful treatment of these tumours includes a multidisciplinary management based on chemotherapy and surgery. While improved outcomes with standardized protocols have been demonstrated in the west, there remains very little data on the outcome of these patients from the Indian subcontinent. We attempt to bridge this gap by presenting our data on the management and outcomes of hepatoblastoma. Methods: A prospectively collected database of over a decade (2010-2022) of children with hepatoblastoma who were treated at our unit was analyzed. Data regarding the clinical presentation, preoperative management, surgical treatment, postoperative course, trend of AFP and outcomes in terms of survival rate and event free survival analyzed. Results: Of the 79 children with hepatoblastoma, 32 &47 were categorized into SIOPEL high and standard risks (4, 21, 34 & 20 were staged as PRETEXT I, II, III & IV respectively). Their median age at diagnosis was 24 months. The median AFP for standard risk group and high risk groups were 197186 ng/ml and 207070 ng/ml respectively. All children received protocoled neoadjuvant chemotherapy before surgery, followed by appropriate course of adjuvant chemotherapy. 68.3%(n=54) underwent liver resection and 25.3%(n=20) underwent liver transplantation. Five children who developed metastatic disease resistant to chemotherapy were not offered surgery. The median follow-up period was 90 months. 5-year overall survival for high risk and standard risk groups were 66.7% and 95.4 % respectively. Conclusions: This experience shows that excellent outcomes can be achieved for hepatoblastoma, even in a resource-challenged setting. Non-transplant surgical resection can be successful in selected patients and, although technically challenging, is associated with a long-term oncological outcome that appears comparable to that obtained with primary liver transplantation.