Clinical Profile of Hepatoblastoma: Experience From a Tertiary Care Centre in a Resource-Limited Setting.

Abstract

BackgroundHepatoblastoma (HB) is a rare neoplasm of the liver, accounting for about 1% of all pediatric cancers. The aim of the present study is to report our experience with HBs over a period of five years from a tertiary center in Eastern India.MethodologyThis is a retrospective observational study. The data of all patients who were diagnosed with HB between August 2015 and December 2020 was reviewed.ResultsTwenty-three patients who were diagnosed and treated for HB at our center were included in the study. Sixteen (69.5%) of them were male. The median age of presentation was 14 (range, 3-58) months. An abdominal lump (n=23, 100%) and abdominal pain (n=11, 47.8%) were the most common presenting symptoms. The median level of serum alpha-fetoprotein at the time of initial evaluation was 8000 (878-1,280,000) ng/dL. The mean size of the largest focus in its largest dimension was 12.03±3.77 cm. The epithelial variant (n=22, 95.7%) was the most common histological subtype. One (4.3%), 10 (43.4%), 11 (47.8%), and one (4.3%) patient were found to have pre-treatment extent of tumor (PRETEXT) stages 1, 2, 3, and 4, respectively. Fifteen (65.2%) children were classified as standard risk and seven (34.7%) children as high risk.All the patients received neoadjuvant chemotherapy (NACT). The most commonly performed surgery was right hepatectomy (n=12, 52.1%). There were three (13%) cases of perioperative mortality. Four postoperative complications developed in three (13%) patients. Four (17.3%) patients developed chemotherapy-related complications. The median duration of follow-up was 31 (range, 0-58) months. Three (13%) patients developed relapses of the disease. Overall, five-year survival in our series was 73.9%.ConclusionThis study shows that the overall outcomes of HB in a resource-limited setting such as ours are good with the adoption of multi-modality treatment. Managing chemotherapy-induced complications and making liver transplantation more feasible will improve the results further.