Hepatoblastoma Arising in a Pigmented β-catenin-activated Hepatocellular Adenoma: Case Report and Review of the Literature.
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.
Departments of *Pathology †Surgery ‡Pediatrics, Stanford University School of Medicine, Stanford, CA Departments of §Pathology ∥Immunology and Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, TX.
Christine Y
CY
Louie
Waldo
W
Concepcion
Joseph K
JK
Park
Arun
A
Rangaswami
Milton J
MJ
Finegold
Florette K
FK
Hazard
D018248:Adenoma, Liver Cell / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000032:analysis; D002675:Child, Preschool; D018197:Hepatoblastoma / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008062:Lipofuscin / Q000378:metabolism; D008113:Liver Neoplasms / Q000473:pathology*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010858:Pigmentation; D051176:beta Catenin / Q000378:metabolism
D014408:Biomarkers, Tumor; C495270:CTNNB1 protein, human; D008062:Lipofuscin; D051176:beta Catenin