Review of Three Cases and the Presentation of a Characteristic Set of Tumor Markers Defining this Tumor

Abstract

This study demonstrates the unique clinical and histologic aspects of fibrolamellar hepatic carcinoma, a rare variant of hepatocellular carcinoma. Three cases are reviewed and an extensive study of immunologic and intracellular substances defining this tumor is presented. Length of survival was considerably longer than typical hepatoma. The cause of death generally is due to a lack of control of the primary tumor. Successful treatment appears to relate to the ability to perform a total excision of the primary hepatic tumor. Chemotherapy should be used only in the presence of metastatic disease. Surgical resection of metastatic disease, unlike the usual hepatocarcinoma, may have some beneficial use. Fibrinogen was found in all tumors. It is possible that this tumor produces fibrinogen to create its unique histologic appearance. Carcinoembryonic antigen is described for the first time in this tumor. Both deposits of alpha-1 antitrypsin and copper were found in most of the tissues studied. The presence and amounts of these substances differ markedly from the common type of hepatoma. This unique composition of intracellular components may both facilitate histologic diagnosis, particularly if the amount of tissue is limited, and give further insight into the etiology of this tumor.

Fibrolamellar Hepatocellular Carcinoma in a Japanese Man: Report of a Case

“…[20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] The younger age and lower coexistence of liver cirrhosis in these Japanese patients with FHCC compared to those of Japanese patients with OHCC, whose mean age is 64.2 years in men and 65.6 years in women, with coexistence of liver cirrhosis in 53.3%, 19 is similar to those reported in Western countries. [3][4][5][6][7][8][9] On the contrary, a male predominance associated with FHCC is coincident with OHCC in Japan, in which the male to female ratio was reported to be 3.0 : 1, 19 and not coincident with FHCC in Western countries. [3][4][5][7][8][9]13 FHCC has a tendency to metastasize to the lymph nodes or peritoneum at a relatively early stage of the disease, when radical surgery could be attempted.…”

“…Edmondson originally described the morphology of FHCC in 1958, 1 after which about 200 cases have been reported, mostly in Western countries, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] where OHCC is a relatively uncommon neoplasm. FHCC is pathologically different from OHCC in that it consists of polygonally shaped plump tumor cells with a highly eosinophilic cytoplasm and bands of fibrous stroma, arranged in parallel lamellae and surrounding clusters of these tumor cells.…”

“…FHCC is pathologically different from OHCC in that it consists of polygonally shaped plump tumor cells with a highly eosinophilic cytoplasm and bands of fibrous stroma, arranged in parallel lamellae and surrounding clusters of these tumor cells. [1][2][3][4][5][6] In Japan, OHCC is one of the most common malignant tumors, with a registration of 16 467 cases during the 2 years from January 1, 1994, until December 31, 1995 according to the Liver Cancer Study Group of Japan. 19 However, only 18 cases of FHCC were reported between 1984 and 1999 (Table 1).…”

“…Clinically, FHCC is not associated with male or female predominance, but the patients are young 3,4,[6][7][8]11,13 have no history of cirrhosis or chronic liver disease, [3][4][5]8,11,13 and do not have abnormal serum tumor markers or hepatitis B antigen. 7,8 The mean age of the 19 FHCC cases in Japan (including ours) was 30.4 years, with a male to female ratio of 2.2 : 1.…”

“…Therefore, even if we had never encountered this type of tumor before, had we possessed better knowledge of its characteristic features, we could possibly have established a correct preoperative diagnosis. α-Fetoprotein and PIVKA-II, which are helpful tumor markers for OHCC, are known not to be associated with FHCC, 6,8,13 although serum neurotensin 15 and serum vitamin B 12 14 are reported to be effective markers. The serum CA125 level, which is a well-established tumor marker for ovarian cancer, has been reported to be helpful in the diagnosis OHCC.…”