A retrospective study on the incidence and outcomes of hepatoblastoma in relation to prognostic indicators at Universitas Academic Hospital, Bloemfontein, South Africa (2000–2022)

Vollständiger Abstract

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Background: Hepatoblastoma (HB) is the most common solid liver cancer in childhood. Globally, survival has significantly improved with advanced treatment modalities. However, survival is poor in low-middle-income countries (LMICs) due to advanced-stage presentation. Aim: The aim was to evaluate the incidence and outcomes of children diagnosed with HB in relation to prognostic indicators which included age, sex, PRE-Treatment EXTent (PRETEXT) staging, metastasis and surgical intervention. Outcomes included survival and mortality. Setting: The research was conducted at the Pediatric Oncology Unit in Universitas Academic Hospital in Bloemfontein, South Africa, which serves as a tertiary referal centre for patients from the Northern Cape, Free State and Lesotho. Methods: We retrospectively analysed medical records of the children with HB diagnosed and treated from 2000 till 2022 at Universitas Academic Hospital Paediatric Oncology Unit. Results: Twenty patients were included with an incidence of 1.2% and a 95% confidence interval (CI) of 0.8%–1.8%. Median age was 2 years old with interquartile range (IQR) of 1 year – 2.97 years. There was a male: female ratio of 1.9:1. All children presented with abdominal distension and elevated alpha fetoprotein (AFP) levels. More than half (55%; n = 11) had PRETEXT IV and 45% (n = 9) had lung metastasis at presentation. Surgical resection was done in 50% (n = 10) and none had liver transplant. The 1 year overall survival (OS) was 55% (n = 11), 2-year OS was 20% (n = 4), and 5 year OS was 10% (n = 2). Higher mortality was noted in children above 3 years old (p = 0.5304), male patients (p = 0.1011), distant metastasis (p = 0.5913), PRETEXT stage IV (p = 0.2621), high risk HB (p = 0.117) and children who did not get surgical resection (p = 0.0867). Conclusion: Survival was negatively impacted by older age, male gender, advanced stage of disease and paucity of surgical intervention. Contribution: This study highlights the factors affecting the survival of children with this rare disease and highlights measures to improve same.