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  • Title: Congenital Hepatoblastoma and Beckwith-Wiedemann Syndrome.
    Author: Zivot A, Edelman M, Glick R, Hong A, Fish JD.
    Journal: J Pediatr Hematol Oncol; 2020 Nov; 42(8):e798-e800. PubMed ID: 31335825.
    Abstract:
    Following the discovery of a fetal hepatic tumor, labor was induced at 38 weeks, and a phenotypically normal female was delivered vaginally. A serum alpha-fetoprotein level at birth was 373,170 ng/mL. Postnatal magnetic resonance imaging confirmed a mass in the right lobe of the liver, and a percutaneous core biopsy revealed an epithelial type hepatoblastoma with predominantly embryonal histology. Methylation testing revealed hypomethylation at imprinting center 2, consistent with a diagnosis of Beckwith-Wiedemann syndrome. This case suggests that Beckwith-Wiedemann syndrome testing should be considered in all patients with hepatoblastoma, even in the absence of other phenotypic stigmata.
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