Clinicopathologic features and survival in fibrolamellar carcinoma: Comparison with conventional hepatocellular carcinoma with and without cirrhosis

Department of Anatomic Pathology, UCSF/VA Medical Center, San Francisco, CA 94121, USA.
Modern Pathology (Impact Factor: 6.19). 12/2005; 18(11):1417-23. DOI: 10.1038/modpathol.3800449
Source: PubMed


Fibrolamellar carcinoma arises in noncirrhotic livers of young individuals and has been considered to be less aggressive than conventional hepatocellular carcinoma. This study compares survival and clinicopathologic features of fibrolamellar carcinoma with hepatocellular carcinoma arising in noncirrhotic and cirrhotic livers. Clinical and pathologic features including age, gender, tumor size, stage and survival were recorded in 20 resected cases of fibrolamellar carcinoma. Survival was compared with resected hepatocellular carcinoma without (n=32) and with cirrhosis (n=30). Proliferative activity was determined by immunohistochemistry for Ki-67. In all, 12 (60%) patients with fibrolamellar carcinoma died during follow-up; the 5-year survival was 45%. Mortality in fibrolamellar carcinoma was higher with metastatic disease at presentation (6/7, 86% vs 5/13, 39%, P=0.06). Age, gender and tumor size did not correlate with survival. The 5-year (45 vs 56%, P=0.4) as well as overall survival (40 vs 56.3%, P=0.3) was similar in fibrolamellar carcinoma and hepatocellular carcinoma without cirrhosis. The 5-year and overall survival in hepatocellular carcinoma with cirrhosis was 27 and 23.3%, respectively, which was not significantly different compared to fibrolamellar carcinoma (P=0.2). Among the cases without metastases at presentation, 5-year survival in fibrolamellar carcinoma (62%) and hepatocellular carcinoma without cirrhosis (57%) was significantly better (P=0.03) than hepatocellular carcinoma with cirrhosis (27%). The mean Ki-67 index was similar in all three groups (P=0.1). In conclusion, fibrolamellar carcinoma is an aggressive neoplasm with 45% 5-year survival and overall mortality of 60%. Nearly half the patients develop lymph node or distant metastasis. The prognosis of fibrolamellar carcinoma is similar to conventional hepatocellular carcinoma. Among nonmetastatic cases, the prognosis is better in fibrolamellar carcinoma and hepatocellular carcinoma without cirrhosis compared to hepatocellular carcinoma with cirrhosis. The better outcome in fibrolamellar carcinoma appears to be due to the absence of cirrhosis rather than its distinct clinicopathologic features.

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    • "FLC is thought to be associated with better outcome than classical HCC, mainly due to the absence of underlying cirrhosis. However, in FLC, metastatic tumour thrombus and direct tumour invasion in the inferior caval vein or the right atrium are often detected at the time of diagnosis and predict poorer survival [6] [7] [8] [9] [10]. Treatment by means of resection, chemotherapy , transarterial chemoembolization and radiation has been described with varying success rates. "
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    Interactive Cardiovascular and Thoracic Surgery 07/2013; 17(5). DOI:10.1093/icvts/ivt334 · 1.16 Impact Factor
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    • "Cholestasis is often present in FLC [10], with canalicular bile plugging the most common cholestatic pattern. Because of the frequent cholestasis, FLC commonly has copper deposition [2, 10, 69, 73, 75, 76]. Of note, copper accumulation is also common in ordinary HCC that is cholestatic and is not a defining feature of FLC [77]. "
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    ABSTRACT: Fibrolamellar carcinomas are a unique type of primary liver cancer. They occur most commonly in children and young adults. Their etiology remains a mystery, as they are not associated with chronic liver disease. Fibrolamellar carcinomas are not indolent tumors, but have an overall better prognosis than typical hepatocellular carcinomas, in part because of the younger age at presentation and the lack of cirrhosis. The most important prognostic feature is whether the tumor is resectable. Histologically, the tumor is made up of large cells that contain abundant mitochondria. The nuclei of the tumor cells have prominent nucleoli. The tumor cells induce the formation of extensive intratumoral fibrosis, which often grows in parallel, or lamellar bands. The tumor cells clearly show hepatocellular features but are also unique in showing both biliary and neuroendocrine differentiation. The uniqueness of fibrolamellar carcinoma extends to their molecular findings. While the genetic abnormalities that lead to fibrolamellar carcinomas are not yet known, studies have shown that they lack mutations in the genes most commonly mutated in typical hepatocellular carcinoma (TP53 and CTNNB1). In this paper, the clinical, pathological, and basic science literature on fibrolamellar carcinoma is comprehensively reviewed. Key areas of needed research are also discussed.
    09/2012; 2012(2):743790. DOI:10.6064/2012/743790
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    Nippon Ika Daigaku Zasshi 03/1993; 60(1):64-6. DOI:10.1272/jnms1923.60.64
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