32. THE SPECTRUM OF PAEDIATRIC LIVER DISEASE SEEN IN A CHILDREN'S HOSPITAL. (February 2015)

• Record Type: Journal Article
• Authors: Leong, M. Y.
• Is Part Of: Pathology. Volume 47(2015)Supplement 1
• Publication Date: 2015-02
• Subjects: Pathology -- Periodicals; 616.0705
• DOI: 10.1097/01.PAT.0000461641.66029.9c
• Languages: English
• ISSNs: 0031-3025

Abstract

Abstract : Background: The spectrum of hepatobiliary disease in infancy and childhood has not been reported in our population. Ours is an 830-bed hospital and academic healthcare institution for women and children in the country and the region. Aims: The aim is to study the histological spectrum of paediatric hepatobiliary diseases encountered in this children's hospital. Methods: The electronic histopathology record was retrospectively reviewed to identify all surgical specimens from liver, including biopsies and resection specimens, from patients under the age of 18 years over a 5-year period (from January 2009 to December 2013). Results: There were 83 liver specimens over the 5-year period. 85.5% of cases were biopsies. The diagnoses of 18 cases (21.7%) were neoplasms, of which 10 were primary liver tumours (hepatoblastoma 8, hepatocellular carcinoma 1, mesenchymal hamartoma 1) and 8 were metastases (metastatic neuroblastoma 6, desmoplastic small round cell tumour 1 and germ cell tumour 1). 51.6% of cases were from infants under the age of 1 year. Non neoplastic cases accounted for 88.4% and included 25 cases of extrahepatic biliary atresia, 5 cases of choledochal cyst and 4 cases of neonatal hepatitis. 24.1% of cases were from children aged 1 to 5 years. Non neoplastic cases accounted for 50% and included 4 cases of choledochal cyst and 2 cases of autoimmune hepatitis. 24.1% of cases were from children aged 6 to 18 years. There were 3 neoplasms and three cases each of choledochal cyst, autoimmune hepatitis and graft-versus-host disease. Conclusions: The most common diagnosis was extrahepatic biliary atresia (30.1%), followed by choledochal cyst (14.5%). 21.7% of cases were neoplasms, with hepatoblastoma being the most common (9.6 %), followed by metastatic neuroblastoma (7.2%).