Primary liver tumours in children in Victoria: incidence and pathology

Abstract

This paper reviews the incidence and pathology of liver tumours in children in the State of Victoria from 1955 to 1987. Seventy-four cases were found and are believed to represent all liver tumours in the State during that time. There were 29 benign and 45 malignant tumours. The benign tumours comprised 13 haemangiomas, 12 mesenchymal hamartomas, and 4 epithelial lesions. The malignant tumours were 30 hepatoblastomas, 8 embryonal sarcomas, 4 hepatocellular carcinomas, and 3 miscellaneous tumours. The haemangiomas were more commonly cavernous. Four were associated with skin lesions and 3 of these were capillary in type. Mesenchymal hamartomas varied from predominantly solid to predominantly cystic. All, however, contained hamartomatous collections of ducts and liver cells irregularly arranged in dense fibrous tissue. The commonest malignant tumour was hepatoblastoma, and there were 15 of predominantly epithelial type and 15 of mixed epithelial and mesenchymal type. The 8 undifferentiated sarcomas of embryonal pattern were characterised by pleomorphism of cells, bizarre giant cells, and extensive mucoid stromal change. Four examples of hepatocellular carcinoma were seen. All were multifocal and no further case has been seen for the last 15 years. The 3 miscellaneous tumours were gastrinoma, rhabdoid tumour, and myofibroblastic tumour of uncertain histogenesis. Incidence was calculated for the period of review. The rate for benign tumours was 0.81 per million children per year and for malignant tumours 1.33 per million children per year. Surgery for hepatobalstoma was shown to be the most important factor in improving survival.