MỘT SỐ ĐẶC ĐIỂM LÂM SÀNG, CẬN LÂM SÀNG VÀ PHÂN LOẠI MÔ BỆNH HỌC CỦA U NGUYÊN BÀO GAN THEO PHÂN LOẠI CÁC KHỐI U TRẺ EM CỦA TỔ CHỨC Y TẾ THẾ GIỚI 2023

Abstract

Introduction: Hepatoblastoma (HB) is a primary malignant liver tumor. It is the most common malignant liver tumor in children, accounting for 90% of malignant liver tumors in children under 5 years of age. In Vietnam, research on HB remains limited, mainly focusing on the clinical features and histopathological classification of HB according to the latest update of the World Health Organization (WHO). Therefore, investigating the clinical and paraclinical characteristics as well as the histopathological classification of HB is necessary. Methods: Cross-sectional descriptive study. Results: At the Vietnam National Children’s Hospital, from January 2016 to January 2025, a total of 140 cases were diagnosed with hepatoblastoma (HB). The mean age at diagnosis was 35,4 ± 39,2 months, with 79,3% of patients being under 5 years old. The male-to-female ratio was 1,46/1. Most tumors were solitary (82,9%) and predominantly located in the right hepatic lobe (44,3%), with a mean tumor size of 8,6 ± 3,8 cm. The mean serum alpha-fetoprotein (AFP) level was markedly elevated at 245600,8 ± 341743 ng/mL. According to the PRETEXT classification, most cases were in stage I and II (61,8%). The most common histopathological subtype was the epithelial type, Pure fetal hepatoblastoma with low mitotic activity (31,4%), followed by Fetal hepatoblastoma, mitotically active (27,9%). The mixed epithelial and mesenchymal type accounted for 12.9%, while other rare subtypes were less frequently observed. Conclusion: Hepatoblastoma in Vietnamese children predominantly occurs at an early age, typically presenting as a solitary tumor in the right hepatic lobe, with markedly elevated AFP levels and mainly early PRETEXT stages. According to the 2023 WHO classification, the epithelial type is predominant, particularly the Pure fetal hepatoblastoma with low mitotic activity and Fetal hepatoblastoma, mitotically active. Multicenter longitudinal studies are needed to correlate histological subtypes with risk stratification, chemotherapy response, and survival outcomes to optimize treatment strategies.