The complex process of diagnosing, staging and treating Liver Tumours in Children is unique in a resource-limited setting. A previous review in South Africa found a low tumour resection rate due to advanced disease. This study intends to explore recent changes in interventions, outcomes and prognostic factors of paediatric liver tumours in Johannesburg, South Africa, in order to improve decision-making and survival. The study aims to describe the experiences and outcomes of paediatric liver tumours at Chris Hani Baragwanath Academic Hospital (CHBAH).
A retrospective review of all patients aged below 16 years, with benign or malignant liver tumours, from 1st January 2005 to 31st December 2017.
Twenty Nine children presented with primary hepatic tumours, 16 (55%) were female; 13 (45%) were male, and the median age was 3 years. Biopsy was done in all cases, there were 15 hepatoblastomas (52%), five hepatocellular carcinomas (17%), three undifferentiated embryonal sarcomas (10%), 2 hamartomas, 2 vascular tumours, and 2 rhabdomyosarcomas. Three cases were PRETEXT stage 1 (11%), 15 were stage 2 (55%), four stage 3 (15%), and four stage 4 (15%). Seven children (23%) had metastasis, four (13%) to the lungs, and three (10%) to the bone. Sixteen cases had surgery, 8 right hepatectomies, 5 extended right hepatectomies, 1 left sectionectomy, and 2 emergencies for rupture. Six cases had inoperable tumours, four demised before surgery, and two defaulted. Overall survival was 48% (14), 38% died (11), and 14%(4) were lost to follow-up. Ten of 15 hepatoblastomas survived (67%), two of three Embryonal Sarcoma’s (66%), one of two hamartoma’s (50%), and one of two vascular tumour’s (50%). Hepatocellular carcinomas and rhabdomyosarcomas both had 0% survival rates.
There was a poor overall survival of 48%, influenced by the large number of inoperable tumours, distant metastases, unfavourable histology, patients demising before surgery or defaulting treatment, necessitating an earlier diagnosis and expedited management.