儿童肝母细胞瘤常见和罕见的组织学变异:病理诊断和文献回顾。

IF 0.8 Q4 GASTROENTEROLOGY & HEPATOLOGY
Gastrointestinal Tumors Pub Date : 2021-04-01 Epub Date: 2021-02-04 DOI:10.1159/000512236
Sushma Bharti, Jyotsna Naresh Bharti, Arvind Sinha, Taruna Yadav
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引用次数: 9

摘要

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,这种肿瘤是散发性的,但可以表现出综合征的相关性。在70-80%的HB病例中,Wnt/β-catenin通路上调。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为上皮型。HB必须与局灶性结节增生、肝细胞腺瘤和肝细胞癌区分开来,而小细胞未分化HB必须与恶性横纹肌样瘤区分开来。组织形态学和免疫组化是诊断不同类型HB的基础。手术后的新辅助化疗是主要的治疗方法。两种确定的HB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组织学变异病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Common and Rare Histological Variants of Hepatoblastoma in Children: A Pathological Diagnosis and Review of the Literature.

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.

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来源期刊
Gastrointestinal Tumors
Gastrointestinal Tumors GASTROENTEROLOGY & HEPATOLOGY-
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审稿时长
17 weeks
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