Dumortier J, Bizollon T, Chevallier M, Ducerf C, Baulieux J, Scoazec J Y, Trepo C
Unité d'Hépatologie, Hôtel-Dieu, Lyon, France.
Gut. 1999 Oct;45(4):622-5. doi: 10.1136/gut.45.4.622.
Hepatoblastoma is an exceptional cause of primary malignant liver tumour in the adult.
The case is reported of an adult patient transplanted for alcoholic cirrhosis complicated by multifocal hepatocellular carcinoma in whom a recurrence in the form of a mixed hepatoblastoma invading the whole transplanted liver developed three months after liver transplantation.
Complete clinical, histopathological, and immunohistochemical data were reviewed.
The recurrent tumour invaded the whole liver. The major component was a mixed hepatoblastoma, with an epithelial component expressing cytokeratin and a mesenchymal component expressing vimentin. The tumour also contained a minor hepatocarcinomatous component expressing alpha fetoprotein. The rapid growth of the tumour prevented any attempt at treatment. Although direct evidence is lacking, the most likely hypothesis to explain the observations is a marked phenotypic change in the initial malignant population at recurrence.
This case supports a possible filiation between hepatocellular carcinoma and hepatoblastoma in adults.
肝母细胞瘤是成人原发性恶性肝肿瘤的罕见病因。
报告了一名因酒精性肝硬化合并多灶性肝细胞癌接受移植的成年患者,肝移植三个月后出现以混合性肝母细胞瘤形式复发并侵犯整个移植肝的情况。
回顾了完整的临床、组织病理学和免疫组化数据。
复发性肿瘤侵犯整个肝脏。主要成分是混合性肝母细胞瘤,上皮成分表达细胞角蛋白,间充质成分表达波形蛋白。肿瘤还含有少量表达甲胎蛋白的肝细胞癌成分。肿瘤的快速生长使任何治疗尝试都无法进行。尽管缺乏直接证据,但解释这些观察结果最可能的假说是复发时初始恶性细胞群发生了明显的表型变化。
该病例支持成人肝细胞癌和肝母细胞瘤之间可能存在谱系关系。