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
{"title":"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。","authors":"Sushma Bharti,&nbsp;Jyotsna Naresh Bharti,&nbsp;Arvind Sinha,&nbsp;Taruna Yadav","doi":"10.1159/000512236","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":45017,"journal":{"name":"Gastrointestinal Tumors","volume":"8 2","pages":"41-46"},"PeriodicalIF":0.8000,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000512236","citationCount":"9","resultStr":"{\"title\":\"Common and Rare Histological Variants of Hepatoblastoma in Children: A Pathological Diagnosis and Review of the Literature.\",\"authors\":\"Sushma Bharti,&nbsp;Jyotsna Naresh Bharti,&nbsp;Arvind Sinha,&nbsp;Taruna Yadav\",\"doi\":\"10.1159/000512236\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":45017,\"journal\":{\"name\":\"Gastrointestinal Tumors\",\"volume\":\"8 2\",\"pages\":\"41-46\"},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2021-04-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1159/000512236\",\"citationCount\":\"9\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Gastrointestinal Tumors\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1159/000512236\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2021/2/4 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Gastrointestinal Tumors","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1159/000512236","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2021/2/4 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
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。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
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.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Gastrointestinal Tumors
Gastrointestinal Tumors GASTROENTEROLOGY & HEPATOLOGY-
自引率
0.00%
发文量
5
审稿时长
17 weeks
期刊最新文献
Investigation of Novel Urinary Biomarkers in Hepatocellular Carcinoma Risk in a Predominantly African American Population: A Case-Control Study. Real-World Outcomes of FLOT versus CROSS Regimens for Patients with Oesophagogastric Cancers. Association between Serum Zinc Levels and Clinicopathological Characteristics in Patients with Gastric Cancer. Mixed Neuroendocrine and Non-Neuroendocrine Neoplasm of Pancreas: What Do We Know, What Have We Learnt? Mast Cell Sarcoma of Small Intestine, Early Diagnosis, and Good Prognosis: An Extremely Rare Case Report and Review of the Literature.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1