Malignant hepatic tumours in children: incidence, clinical features and aetiology

Abstract

Congenital defects and other disorders have been reported in association with malignant liver tumours. In order to assess their significance, a population-based survey was undertaken on children aged less than 15 years diagnosed with malignant liver tumours during the 30 years 1957-1986. The cases were identified from information collected by the West Midlands Regional Children's Tumour Registry. Pertinent data were extracted from their clinical records, and the original biopsy and any necropsy material were reviewed by a panel of three paediatric pathologists. Of the 50 eligible cases registered, eight were excluded because histology review showed that they had non-malignant conditions (3) or malignancies of extrahepatic origin (4) or because no pathological material was available (1). The diagnoses in the remaining 42 cases were hepatoblastoma (27), hepatocellular carcinoma (3), rhabdomyosarcoma (6), rhabdoid tumour (3) and yolk sac tumour (3). The incidence of primary malignant liver tumours was 1.20 per 10(6) person years and that of the hepatoblastoma sub-group was 0.77 (average childhood population of the West Midlands for the time period being 1,166,500). The presenting clinical, radiological and biochemical features were similar to those reported in other series and the ethnic and social class distributions were unremarkable compared with the local population. Congenital defects or other possibly related features were present in nine (21%) patients. Our results, taken with other reports, suggest that hepatoblastoma is a malignant tumour related to maldevelopment, possibly associated with 11p or 5q mutations, whereas hepatocellular carcinoma is more usually a complication of metabolic and other disorders which lead to cirrhosis.

Differential diagnosis and management of liver tumors in infants

“…Maternal smoking, parental occupation and genetic susceptibility (gene MPO, NQ01, SULT, IGF-2 and so on) have been associated with HB and recent studies provide support for an increased risk of HB in low (1500-2500 g) and very low (< 1500 g), birth weight infants, in which HB is diagnosed at older ages and in more advanced stages than HB cases of normal birth weight. Neonatal therapies including supplemental oxygen, phototherapy, administration of numerous drugs, total parenteral nutrition and blood transfusions may play a role in the development of HB [36] . An infant with HB usually presents with an abdominal mass often detected by a parent.…”

Overexpression of Human Dickkopf-1, an Antagonist of wingless/WNT Signaling, in Human Hepatoblastomas and Wilms' Tumors

“…They are rare tumors with an incidence of about one new case per one million children per year (Mann et al, 1990). Little is known of the molecular pathogenesis of HBs.…”

Blocking the hedgehog pathway inhibits hepatoblastoma growth

“…1 The incidence in Western countries is estimated to be approximately one HB case per million children, 2 with an increasing trend corresponding to an improved survival of very-low-birth-weight babies. 3 Pathohistologically, HB resembles various stages of the developing liver, showing malignant epithelial cells with fetal or embryonal hepatic differentiation and foci of primitive blastemal cells.…”