Hepatoblastoma Arising in a Pigmented β-catenin-activated Hepatocellular Adenoma
Hepatoblastoma is the most common malignant liver tumor in childhood. It has been associated with a variety of constitutional syndromes and gene mutations. However, there are very few reports of associations with pediatric hepatocellular adenomas (HCAs) and no reported associations
with pigmented HCAs (P-HCAs). We present a unique case of hepatoblastoma arising in a background of 2 β-catenin-activated HCAs, one of which is pigmented, in a 4-year-old child. The gross, histologic, and immunohistochemical features are described for each tumor. In addition, the literature
is reviewed with specific emphasis on the clinical and pathologic features of B-HCAs. Although the potential of β-catenin-activated HCAs to progress to hepatocellular carcinoma has been well documented, there are very few reports of their potential to progress to hepatoblastoma. We not
only present such a case, but, to our knowledge, we also present the first case of a P-HCA in a child.
Keywords: hepatic adenoma; hepatoblastoma; pigmented; β-catenin
Document Type: Case Report
Affiliations: 1: Departments of Pathology 2: Surgery 3: Pediatrics, Stanford University School of Medicine, Stanford, CA 4: Departments of Pathology, Immunology and Pediatrics, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX 5: Departments of Pathology, Pediatrics, Stanford University School of Medicine, Stanford, CA
Publication date: 01 July 2016
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