• M Klimpfinger, G Jatzko, A Trink, H J Samec, and A Gaugg.
• Wien. Klin. Wochenschr. 1987 Jul 17;99(14):505-9.

AbstractThis paper presents the case report of the coincidental presence of an adenocarcinoma of the rectum and fibrolamellar hepatocellular carcinoma in a 76 year-old man. The tumours were successfully removed by simultaneous resection. The characteristics of hepatocellular carcinoma of the fibrolamellar type are discussed in detail on the basis of the presented case and a survey of the literature. This variant of hepatocellular carcinoma is predominantly found in younger patients. It appears in non-cirrhotic livers and is preferentially localized in the right lobe. Histologically, fibrolamellar hepatocellular carcinoma is characterized by large eosinophilic tumour cells. The cells show trabecular or solid arrangement separated by fibrous septa. Cytoplasmic globules are often found in the tumour cells. In the literature, fibrinogen, CEA, alpha-1-antitrypsin, copper binding protein and copper have been demonstrated in the tumour cells by immunohistochemistry and histochemistry. The fibronectin content is increased in the tumour and seems to correlate with a higher degree of differentiation, as well as a better prognosis. The serum alpha-fetoprotein levels of patients with fibrolamellar carcinomas are normal, in contrast to those in patients with other types of hepatocellular carcinomas. The serum vitamin B12 binding-capacity, as well as neurotensin concentrations are increased. Patients with fibrolamellar carcinomas have a much better prognosis than patients with ordinary hepatocellular carcinomas because of the earlier onset of symptoms, slower tumour progression with late metastasis, and better operability. Two-year survivals of 82% and five-year survivals of 63% have been reported. The prognosis is also better after total hepatectomy followed by liver transplantation.

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