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Comparison of Tumour Characteristics and Outcomes in Paediatric Conventional Versus Fibrolamellar Hepatocellular Carcinoma: Tertiary Centre Experience
Moath Alarabiyat1, Darius F. Mirza1, David Hobin2, Evelyn Ong1, Ravi Marudanayagam1, Khalid Sharif11The Liver Unit, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom, B4 6NH; The Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom, B15 2GW2The Liver Unit, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom, B4 6NH
Objectives: Hepatocellular carcinoma (HCC) in the paediatric age group is a rare and unique disease, with limited evidence to guide treatment decisions. The two main histological subtypes, conventional HCC (cHCC) and fibrolamellar HCC (FL-HCC), differ in their natural history and clinical characteristics. Whether the absence of underlying cirrhosis in FL-HCC confers a survival advantage remains unclear due to inconclusive evidence.
Methods: A retrospective analysis was conducted at a tertiary centre in the United Kingdom, evaluating paediatric patients with HCC referred between 1994 and 2022. Subgroup analysis compared outcomes between cHCC and FL-HCC. Kaplan–Meier analysis was used to compare survival, and Cox regression analysis was performed to identify factors associated with worse survival.
Results: A total of 27 patients were included (cHCC = 17, FL-HCC = 10). The median age was 11 years (cHCC = 9 years, FL-HCC = 14 years), with a male-to-female ratio of 2.5: 1 (M = 20, F = 8). Cirrhosis was present in 8 of 17 evaluable cHCC cases and absent in all FL-HCC cases. Surgical treatment was undertaken in 15 cHCC patients (hepatectomy = 3, transplantation = 12) and 8 FL-HCC patients (hepatectomy = 6, transplantation = 2). Median overall survival was 29 months for cHCC and 42 months for FL-HCC (P = 0.580). Median recurrence-free survival was 29 months for cHCC and 31.5 months for FL-HCC (P = 0.395). Cox regression identified PRETEXT stage as an independent prognostic factor for poorer survival.
Conclusion: Although FL-HCC occurs in non-cirrhotic livers and presents in older children, no significant survival advantage was observed compared to conventional HCC. Tumour extent, as reflected by PRETEXT stage, remains a key prognostic factor. Further multicentre studies are needed to better define optimal treatment strategies for paediatric HCC.
Keywords: Paediatric Hepatocellular Carcinoma, Conventional Hepatocellular Carcinoma, Fibrolamellar Hepatoclleular Carcino-ma, Liver Transplantation
Manuscript Language: English