Khoo J J, Clouston A
Department of Pathology, Hospital Sultanah Aminah, Johor Bahru, Malaysia.
Malays J Pathol. 2001 Dec;23(2):115-8.
A 6-year-old Malay boy presented with fever and abdominal pain for 2 months. Computerised tomography showed a nodular mass in the left lobe of the liver. There was also portal vein thrombosis on the left side. Serum alpha-fetoprotein was not elevated and Hepatitis B antigen was negative. Biopsy of the liver mass led to a histological diagnosis of fibrolamellar hepatocellular carcinoma. In view of extensive tumour involvement, he could not be operated on but was treated with chemotherapy. However, the tumour did not respond. While this is expected for fibrolamellar hepatocellular carcinoma, the possibility of the tumour having a component of ordinary hepatocellular carcinoma could not be excluded as the tumour was not resected. Fibrolamellar hepatocellular carcinoma is a rare histological subtype of hepatocellular carcinoma, associated with a better prognosis. It affects the younger age group and has no association with cirrhosis, hepatitis B virus infection or exposure to oral contraceptives, all of which are implicated in ordinary hepatocellular carcinoma. Serum alpha-fetoprotein level is usually within normal limits and other laboratory values are not contributory to the diagnosis. The diagnosis is usually suggested by radiographic studies viz. CT scan of the abdomen, which would show an irregular non-homogenous mass in the liver, and confirmed by histological examination. The most characteristic microscopical feature is fibrosis arranged in a lamellar fashion around polygonal and deeply eosinophilic neoplastic hepatocytes.
一名6岁马来男孩出现发热和腹痛2个月。计算机断层扫描显示肝脏左叶有一个结节性肿块。左侧还有门静脉血栓形成。血清甲胎蛋白未升高,乙肝抗原阴性。肝脏肿块活检得出纤维板层状肝细胞癌的组织学诊断。鉴于肿瘤广泛累及,他无法接受手术,但接受了化疗。然而,肿瘤没有反应。虽然纤维板层状肝细胞癌出现这种情况是预期的,但由于肿瘤未切除,不能排除肿瘤含有普通肝细胞癌成分的可能性。纤维板层状肝细胞癌是肝细胞癌一种罕见的组织学亚型,预后较好。它影响较年轻的年龄组,与肝硬化、乙肝病毒感染或口服避孕药接触均无关,而这些因素在普通肝细胞癌中都有涉及。血清甲胎蛋白水平通常在正常范围内,其他实验室检查结果对诊断无帮助。诊断通常由影像学检查提示,即腹部CT扫描,其会显示肝脏内不规则的不均匀肿块,并通过组织学检查得以证实。最具特征性的显微镜下特征是围绕多边形且嗜酸性深染的肿瘤性肝细胞呈板层状排列的纤维化。