Bharti Sushma, Bharti Jyotsna Naresh, Sinha Arvind, Yadav Taruna
Department of Pathology, All India Institute of Medical Sciences, Jodhpur, India.
Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, India.
Gastrointest Tumors. 2021 Apr;8(2):41-46. doi: 10.1159/000512236. Epub 2021 Feb 4.
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.
肝母细胞瘤(HB)是一种罕见肿瘤,但却是儿童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,这种肿瘤为散发性,但可与某种综合征相关。70 - 80%的HB病例中可出现Wnt/β - 连环蛋白信号通路的上调。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:上皮型和间叶型。大多数HB为上皮型。HB必须与局灶性结节性增生、肝细胞腺瘤和肝细胞癌相鉴别,而小细胞未分化型HB则需与恶性横纹肌样瘤相鉴别。组织形态学和免疫组化对不同类型HB的诊断至关重要。新辅助化疗后手术是主要治疗方法。HB两个公认的预后因素是分期和组织学类型。在此,我们报告一组HB常见和罕见组织学变异的病例系列。