Publication

Abstract :

Introduction : Hepatoblastoma is a rare pediatric liver tumor. Advances in imaging/surgical techniques and use of neoadjuvant chemotherapy (NACT) in recent times have resulted in improved survival of children with hepatoblastoma. Yet it has dismal prognosis in some children. Unlike other pediatric malignant tumors, pathological tumor regression grading in hepatoblastoma following NACT is not in routine practice. Assessing tumor-induced maturation and delineating it from non-neoplastic liver at resection margin are often challenging in this setting.

Objective : We aim to describe the clinicopathological spectrum of hepatoblastoma encountered in our center with emphasis on exploring the role of grading the therapy-induced changes by correlating with existing prognostic factors and patient survival.

Materials and Methods :  All cases of hepatoblastoma having undergone resection after NACT over 9 years were included. Pathology slides (hematoxylin and eosin/immunohistochemistry) were reviewed. Therapy-related changes were scored and compared with pretreatment extent (PRETEXT)/posttreatment extent (POSTTEXT) staging, alpha fetoprotein (AFP) levels, and patient survival.

Results : A total of 15 children diagnosed with hepatoblastoma were included in the study. The median age of diagnosis was 10 months. PRETEXT III was the commonest stage and fetal variant was the commonest histological subtype. Fibrosis, necrosis, maturation, calcification, and ductular reaction were the therapy-induced changes encountered in 93, 80, 60, 53 and 33% cases, respectively. Higher percentage of therapy-induced changes was associated with good prognosis and better survival. Glypican-3 positivity delineated tumor-induced maturation from the non-neoplastic liver.

Conclusion : This study describes the spectrum of hepatoblastoma at a single center and emphasizes that grading therapy-induced changes may have a significant role in patient prognosis and guide further treatment interventions for effective management of patients. Glypican-3 eases microscopic assessment of resection margins in the presence of therapy-induced maturation.