Dehner L P, Manivel J C
Department of Laboratory Medicine and Pathology, University of Minnesota Medical School and Hospital, Minneapolis 55455.
Am J Pediatr Hematol Oncol. 1988 Winter;10(4):301-7.
Hepatoblastoma is universally recognized and appreciated as one of the unique embryonic tumors of childhood whose morphologic features attempt to recapitulate some of the developmental aspects of the liver. One of the earliest reports referred to this tumor as a teratoma, and it was not until 60 years later that the hepatoblastoma was clearly differentiated from the hepatocellular carcinoma. With the success of the National Wilms' Tumor Study in the recognition and definition of prognostically favorable and unfavorable pathologic types of Wilms' tumor, efforts have been made in this same direction with hepatoblastoma and other malignancies in the pediatric-age population. This review analyzes the progress that has been made in the delineation of morphologic subtypes of hepatoblastoma and their reliability as indicators of prognosis. We conclude that the data are somewhat contradictory about the significance of pure fetal histology as a favorable factor. There are presently too few observations on the so-called macrotrabecular hepatoblastoma to be certain whether it is prognostically unfavorable or not, but general agreement exists about the poor prognosis associated with the rare small cell or anaplastic hepatoblastoma. It is important for clinicians and pathologists to remember that the liver is also the site of other poorly differentiated and primitive-appearing neoplasms that are distinctive entities from hepatoblastoma.
肝母细胞瘤被公认为是儿童期独特的胚胎性肿瘤之一,其形态学特征试图重现肝脏的一些发育过程。最早的报告之一将这种肿瘤称为畸胎瘤,直到60年后肝母细胞瘤才与肝细胞癌明确区分开来。随着国家肾母细胞瘤研究在识别和定义肾母细胞瘤预后良好和不良的病理类型方面取得成功,人们也朝着同样的方向对儿童期人群中的肝母细胞瘤及其他恶性肿瘤进行了研究。本综述分析了在肝母细胞瘤形态学亚型的划分及其作为预后指标的可靠性方面所取得的进展。我们得出的结论是,关于单纯胎儿组织学作为有利因素的意义,数据有些相互矛盾。目前关于所谓的大梁型肝母细胞瘤的观察数据太少,无法确定其预后是否不良,但对于罕见的小细胞或间变性肝母细胞瘤预后不良这一点,人们已达成普遍共识。临床医生和病理学家必须牢记,肝脏也是其他低分化且外观原始的肿瘤的发生部位,这些肿瘤是与肝母细胞瘤不同的独特实体。