Which pediatric liver tumor is strongly associated with Beckwith-Wiedemann syndrome?

Which pediatric liver tumor is strongly associated with Beckwith-Wiedemann syndrome?

• A. Hepatocellular Carcinoma
• B. Fibrolamellar carcinoma
• C. Angiosarcoma
• D. Hepatoblastoma

Answer: (D)

Beckwith-Wiedemann syndrome predisposes children to embryonal tumors, especially hepatoblastoma. This overgrowth syndrome involves imprinting abnormalities on chromosome 11p15, leading to overexpression of growth factors like IGF2, which increases the risk of early-life tumors. Hepatoblastoma is the most common malignant liver tumor in children and typically appears in those under 3 years old, often with elevated AFP. The other tumors listed have different age patterns or etiologies and are not as strongly linked to Beckwith-Wiedemann syndrome. So the liver tumor most strongly associated with Beckwith-Wiedemann is hepatoblastoma.

Beckwith-Wiedemann syndrome predisposes children to embryonal tumors, especially hepatoblastoma. This overgrowth syndrome involves imprinting abnormalities on chromosome 11p15, leading to overexpression of growth factors like IGF2, which increases the risk of early-life tumors. Hepatoblastoma is the most common malignant liver tumor in children and typically appears in those under 3 years old, often with elevated AFP. The other tumors listed have different age patterns or etiologies and are not as strongly linked to Beckwith-Wiedemann syndrome. So the liver tumor most strongly associated with Beckwith-Wiedemann is hepatoblastoma.