Editorial Article

Clinic pathological profile of Hepatoblastoma: an experience from a tertiary care centre in India

Dr. B. Archana,
SRU , India

Primary hepatic malignancies account for 0.5-2% of all solid tumours in childhood. Hepatoblastoma is the most common primary hepatic malignancy in the pediatric age group.

Dr. B. Archana carried her MBBS in Rajiv Gandhi University of Health Sciences, Karnataka and Diploma in Emergency Medicine from the Royal College of General Practitioners, UK. Presently she is pursuing final year M.D Pathology and also working as a Junior Resident in the Department of Pathology, Sri Ramachandra Medical College & Research Institute, India.

Introduction: Primary hepatic malignancies account for 0.5-2% of all solid tumours in childhood. Hepatoblastoma is the most common primary hepatic malignancy in the pediatric age group. Advances in pathological evaluation, imaging, risk stratification, neoadjuvant chemotherapy and surgery including transplantation have improved survival of these children in the western countries. However, successful outcome in developing countries like India with limited resources poses great challenges to the clinician and the family.
Materials and methods: Retrospective review of 10 children diagnosed to have hepatoblastoma between Jan 2010 and Dec 2015 in our institution. Clinical, laboratory, radiological, histopathological, treatment and outcome data were collected and analysed.
Results: The median age of these children at diagnosis was 11 months and only 1 child was premature at birth. Most children presented with abdominal distension. One child had lung metastasis at presentation. All except one presented with thrombocytosis. Nine of the 10 children had elevated serum AFP levels. One child with AFP <100ng/ml died within 3 months. The histological types were fetal, embryonal, macrotrabecular and mixed epithelial mesenchymal types. One out of 6 children with less favourable histology died. SIOPEL risk stratification done showed 6 children to be of standard risk and 4 to be of high risk. Three children had PRETEXT 1,3 PRETEXT 2,1 PRETEXT 3 and 3 PRETEXT 4.Out of 6 who received treatment(PLADO+/- surgery),1 died and 1 has been planned for transplantion.
Conclusion: Our study is significant as very few studies have been published from India and other developing countries and fewer with information on PRETEXT staging, risk status and histological favourability. In developing countries with limited resources and low socioeconomic status, it is important to have a multidisciplinary team approach and tailor treatment in order to manage these patients effectively and improve the overall survival.

Published: 11 May 2017