Sergi Consolato M, Rojas-Vasquez Marta, Noga Michelle, Dicken Bryan
Anatomic Pathology Division, Children's Hospital of Eastern Ontario, Ottawa, ON K1H 8L1, Canada.
Department of Lab. Medicine and Pathology, Stollery Children's Hospital, University of Alberta, Edmonton, AB T6G 2B7, Canada.
Children (Basel). 2022 Apr 15;9(4):565. doi: 10.3390/children9040565.
Liver neoplasms are quite rare in childhood. They often involve 6.7 cases per 10 million children aged 18 years or younger. Hepatoblastoma (HB) is the most frequent tumor, but this neoplasm's rarity points essentially to the difficulty of performing biologic studies and large-scale therapeutic trials. On the pathological ground, HB is separated into an entirely epithelial neoplasm or a mixed neoplasm with epithelial and mesenchymal components. This last category has been further subdivided into harboring teratoid features or not. The 'teratoid' HB includes a mixture of components with heterologous origin. The heterologous components include neuroectoderm, endoderm, or melanin-holding cells with or without mesenchymal components. The most important criterium for the teratoid component is neuroepithelium, melanin, and, more recently, a yolk-sac-like component and neuroendocrine components. The mesenchymal components include muscle, osteoid, and cartilage, which are most often observed mainly in 'teratoid' neoplasms. The teratoid component or mesenchymal components are diagnosed with biopsies. They appear more prominent after chemotherapy due to the response and shrinkage of epithelial elements and non- or low-responsive components of mixed HB. This review focuses on the clinical, radiological, and pathological findings of HB with teratoid features.
肝脏肿瘤在儿童时期相当罕见。每1000万18岁及以下儿童中通常有6.7例发病。肝母细胞瘤（HB）是最常见的肿瘤，但这种肿瘤的罕见性本质上表明进行生物学研究和大规模治疗试验存在困难。在病理学上，HB被分为完全上皮性肿瘤或具有上皮和间充质成分的混合性肿瘤。后一类又进一步细分为有无畸胎样特征。“畸胎样”HB包括具有异源起源的成分混合物。异源成分包括神经外胚层、内胚层或含黑色素的细胞，有无间充质成分均可。畸胎样成分的最重要标准是神经上皮、黑色素，以及最近发现的卵黄囊样成分和神经内分泌成分。间充质成分包括肌肉、类骨质和软骨，最常主要在“畸胎样”肿瘤中观察到。畸胎样成分或间充质成分通过活检诊断。由于混合性HB上皮成分的反应和萎缩以及无反应或低反应成分，化疗后它们显得更为突出。本综述重点关注具有畸胎样特征的HB的临床、影像学和病理学表现。
