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Hepatoblastoma Arising in a Pigmented ß-catenin-activated Hepatocellular Adenoma: Case Report and Review of the Literature. American journal of surgical pathology Louie, C. Y., Concepcion, W., Park, J. K., Rangaswami, A., Finegold, M. J., Hazard, F. K. 2016; 40 (7): 998-1003

Abstract

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 ß-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 ß-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.

View details for DOI 10.1097/PAS.0000000000000652

View details for PubMedID 27096257