Lack E E, Neave C, Vawter G F
Am J Surg Pathol. 1982 Dec;6(8):693-705.
The clinical and pathologic features of 54 children with hepatoblastoma are reviewed. Their average age at diagnosis was 17 months (range: 4 months-4 1/2 years) and there was a predilection for males in a ratio of nearly 2:1. Unusual manifestations included isosexual precocity (two cases), hemihypertrophy and Budd-Chiari syndrome (one case), and synchronous Wilms' tumor (one case). Thirty-two of 49 conventional hepatoblastomas were subclassified as epithelial type (19 predominantly embryonal, 13 predominantly fetal), and 12 had mixed epithelial-mesenchymal histology. Pathologic material in five cases was too limited for reliable subclassification. Hepatoblastomas classified as anaplastic type had more primitive histology with a predominance of small- to medium-sized cells in diffuse sheets. The overall mortality was 76% and the average duration of disease from diagnosis to death was 8 months (range: 1 month-27 months). Complete surgical resection remains the key treatment for achieving long-term survival and was attempted in 33 of the 54 children (61%). There were 13 long-term survivors followed for an average of 9 years. The most common type of hepatoblastoma in these children was epithelial with a predominantly fetal pattern. None of the children with anaplastic tumors survived.
回顾了54例儿童肝母细胞瘤的临床和病理特征。他们诊断时的平均年龄为17个月（范围：4个月至4岁半），男性更易患病，男女比例接近2:1。不寻常的表现包括同性性早熟（2例）、偏侧肥大和布加综合征（1例）以及同步性肾母细胞瘤（1例）。49例传统肝母细胞瘤中，32例被分类为上皮型（19例主要为胚胎型，13例主要为胎儿型），12例具有上皮-间叶混合组织学特征。5例的病理材料有限，无法进行可靠的亚分类。分类为间变型的肝母细胞瘤组织学更原始，以弥漫片状的中小细胞为主。总体死亡率为76%，从诊断到死亡的平均病程为8个月（范围：1个月至27个月）。完整的手术切除仍然是实现长期生存的关键治疗方法，54例儿童中有33例（61%）尝试了手术切除。有13例长期存活者，平均随访9年。这些儿童中最常见的肝母细胞瘤类型是主要为胎儿型的上皮型。间变型肿瘤的儿童无一存活。
