A Clinicopathological Study of Paediatric Liver Tumours in a Tertiary Care Hospital

Abstract

Introduction: Paediatric primary liver tumours are the third largest group of solid abdominal neoplasms in children next to neuroblastoma and Wilms tumour, accounting for about 0.5% to 2% of all paediatric neoplasms, in which hepatoblastoma is the most common. Aim: The present study was done to estimate the incidence of paediatric liver tumours over a period of five years and also, to study the clinical behaviour, alpha-fetoprotein correlation and histopathological features of paediatric liver tumours. Materials and Methods: The details of patients treated for paediatric liver tumours for a period of five years were retrospectively retrieved from the tumour board and medical records. The gross features and all the slides were reviewed and the pathologic diagnosis was confirmed and clinicopathological correlation was then done. Results: A total of 39 paediatric liver tumours were detected during five years, of which 32 (82%) were malignant and seven (18%) were benign with a male to female ratio of 1:1.1. Hepatoblastoma was the most common liver tumour accounting for 28 cases (71.8%), of which 25 cases (89.4%) were of pure epithelial type. The second most common primary tumour was epithelioid haemangioendothelioma with six cases (15.4%) with female preponderance, followed by two cases each of hepatocellular carcinoma and undifferentiated embryonal sarcoma and one case of hepatocellular adenoma. Serum AFP level was increased in hepatoblastoma and in hepatocellular carcinoma, normal in hepatocellular adenoma and embryonal sarcoma. Serum AFP level was increased in few cases of epithelioid haemangioendothelioma. Conclusion: The spectrum of liver tumours in children is different from that in the adults. Hepatoblastoma is the most common paediatric liver tumour, followed by epithelioid haemangioendothelioma. Through better understanding of pathological diagnosis, refined surgical staging, newer and more effective radiological techniques and standardized multimodal therapies, a substantial number of children diagnosed with this highly malignant tumour can expect to survive the disease.

Keywords

• epithelioid haemangioendothelioma
• hepatic tumour
• hepatoblastoma
• hepatocellular carcinoma