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首页> 外文期刊>American Journal of Surgical Pathology >Hepatoblastoma Arising in a Pigmented -catenin-activated Hepatocellular Adenoma: Case Report and Review of the Literature
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Hepatoblastoma Arising in a Pigmented -catenin-activated Hepatocellular Adenoma: Case Report and Review of the Literature

机译：色素连环蛋白激活的肝细胞腺瘤引起的肝母细胞瘤：病例报告和文献复习

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摘要

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 -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 -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.
机译：肝母细胞瘤是儿童期最常见的恶性肝肿瘤。它已经与多种体质综合症和基因突变有关。然而，很少有与小儿肝细胞腺瘤（HCA）相关的报道，也没有与色素性HCA（P-HCA）相关的报道。我们介绍了一个4岁儿童在2 -catenin激活的HCAs背景下发生的肝母细胞瘤的独特案例，其中一个是色素沉着的。描述了每种肿瘤的总体，组织学和免疫组化特征。此外，对文献进行了特别的回顾，重点是B-HCA的临床和病理特征。尽管已经充分证明了-catenin激活的HCAs可能发展为肝细胞癌的潜力，但很少有报道表明它们可能发展为肝母细胞瘤。我们不仅提出这种情况，而且据我们所知，我们还提出了儿童中的第一例P-HCA病例。

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