Stocker J T
Armed Forces Institute of Pathology, Washington, DC 20306-6000.
Semin Diagn Pathol. 1994 May;11(2):136-43.
Hepatoblastoma is the most frequently occurring liver tumor in children, accounting for over 25% pediatric hepatic tumors and nearly 50% of those that are malignant. Histologically, the tumor can be divided into the following six patterns: (1) fetal epithelial; (2) embryonal and fetal epithelial; (3) macrotrabecular; (4) small cell undifferentiated; and (5) mixed epithelial and mesenchymal type with teratoid features or (6) without teratoid features. Immunohistochemical studies display a wide variety of immunostaining with monoclonal antibodies particularly those specific for epithelial-derived components. Tumor cytogenetics show a high incidence of trisomy 20 and trisomy of all or part of chromosome 2. The developing liver displays many features similar to those seen in hepatoblastoma, including uniform hepatocytes and cords two cells thick separated by sinusoids displaying hematopoiesis. Hepatoblastomas display only minimal ductular differentiation, similar to the fetal development of the liver that does not display significant ductular development until well into the second trimester.
肝母细胞瘤是儿童中最常见的肝脏肿瘤，占小儿肝脏肿瘤的25%以上，占恶性肝脏肿瘤的近50%。从组织学上看，该肿瘤可分为以下六种类型：（1）胎儿上皮型；（2）胚胎型和胎儿上皮型；（3）大梁型；（4）小细胞未分化型；以及（5）具有畸胎样特征的混合上皮和间充质型或（6）无畸胎样特征型。免疫组织化学研究显示，单克隆抗体，尤其是那些针对上皮衍生成分的特异性抗体，呈现出多种免疫染色。肿瘤细胞遗传学显示20号三体以及2号染色体全部或部分三体的发生率很高。发育中的肝脏表现出许多与肝母细胞瘤相似的特征，包括均匀的肝细胞和由显示造血功能的窦状隙分隔的两层细胞厚的索状结构。肝母细胞瘤仅表现出极少的胆管分化，类似于肝脏的胎儿发育，直到妊娠中期才会出现明显的胆管发育。
