Hepatoblastoma (HB) is a rare tumor, but it is the most common primary liver malignancy in children and comprised of approximately 1% of all pediatric malignancies. Mostly, this tumor is sporadic in nature but can show a syndrome association. Upregulation in Wnt/β-catenin pathway can be there in 70–80% cases of HB. Most often present as abdominal mass and has a raised alpha-fetoprotein levels. Distant metastasis usually occurs in the lungs. HB is classified into 2 broad categories: epithelial and mesenchymal type. The majority of HB are epithelial type. The HB must be distinguished from focal nodular hyperplasia, hepatocellular adenoma, and hepatocellular carcinoma, while small-cell undifferentiated HB from the malignant rhabdoid tumor. The histomorphology and immunohistochemistry are essential for the diagnosis of different HB. The neoadjuvant chemotherapy followed by surgery is the mainstay of the treatment. The 2 well-established prognostic factors of HB are stage and histological type. Herein, we report a case series of common and rare histological variants of HB.
Gastrointest Tumors

中文翻译：

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儿童肝母细胞瘤常见和罕见的组织学变异：病理诊断和文献复习

肝母细胞瘤 (HB) 是一种罕见的肿瘤，但它是儿童中最常见的原发性肝脏恶性肿瘤，约占所有儿科恶性肿瘤的 1%。大多数情况下，这种肿瘤本质上是散发性的，但可以显示出与综合征有关。Wnt/β-catenin 通路上调可出现在 70-80% 的 HB 病例中。最常表现为腹部肿块，甲胎蛋白水平升高。远处转移通常发生在肺部。HB分为两大类：上皮型和间充质型。大多数HB是上皮型。HB 必须与局灶性结节增生、肝细胞腺瘤和肝细胞癌相鉴别，而小细胞未分化 HB 与恶性横纹肌样瘤相鉴别。组织形态学和免疫组织化学对不同 HB 的诊断至关重要。新辅助化疗后手术是治疗的主要方式。HB 的 2 个公认的预后因素是分期和组织学类型。在此，我们报告了一系列常见和罕见的 HB 组织学变异。
胃肠道肿瘤