Fibrolamellar Hepatocellular Carcinoma

Department of Gastroenterology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China.
The American Journal of Gastroenterology (Impact Factor: 9.21). 08/2009; 104(10):2617-24; quiz 2625. DOI: 10.1038/ajg.2009.440
Source: PubMed

ABSTRACT Fibrolamellar hepatocellular carcinoma (FHLCC) generally occurs in young individuals lacking a background of chronic liver disease and other risk factors for hepatocellular carcinoma. The clinical presentations of FLHCC are generally nonspecific, and the alpha-fetoprotein level is typically within the normal range in most cases. Imaging studies have a major role in clinical diagnosis, but pathology is the gold standard in confirming diagnosis. Pathological characteristics of FLHCC include the presence of tumor cells with a deeply eosinophilic cytoplasm and macronucleoli surrounded by abundant fibrous bands. The most effective treatment for FLHCC is aggressive surgical resection. This comprehensive literature review gives a full account of the clinical, pathological, and molecular features of FLHCC.

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    • "Surgical resection is the mainstay of treatment. Compared with conventional HCC, this tumor does not respond well to chemotherapy [2]. Based on these features, FLHCC is now considered as a distinct form of liver cancer. "
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    ABSTRACT: fibrolamellar hepatocellular carcinoma (FLHCC) has a better prognosis than conventional hepatocellular carcinoma. Nevertheless, FLHCC has a propensity to recur with limited responsiveness to chemotherapy. The purpose of this study was to provide insight into the cell cycle biology of FLHCC, as it relates to FLHCC's relatively indolent nature and lack of chemoresponsiveness. in seven cases of FLHCC, we assessed: 1. immunoexpression of protein analytes indicating cell cycle progression including Ki-67 (G1, S, G2 and M phases) and S-phase kinase-associated protein (Skp) 2 along with the mitotic index (MI); 2.immunoreactivity for cyclin-dependent kinase inhibitors of cell cycle progression from G1 to S phase, p27Kip1 and p16INK4. the mean percentage of Ki-67 nuclear positivity in neoplastic hepatocytes ranged from 1.0% to 29.7%. Nuclear Skp2 immunoexpression was not observed in any of the cases. The mitotic index was very low (0-1 mitotic figure / 10 high-power fields). All cases showed moderate to strong nuclear p16INK4 positivity (diffuse in five and focal in two). Contras-tively, the adjacent non-neoplastic hepatocytes expressed only mild (2 cases) to no (3 cases) p16INK4. our analysis has revealed that cell cycle arrest in FLHCC occurs in G0G1 phase and is associated with overexpression of the cell cycle regulator, p16INK4 in tumoral cell nuclei compared with non-neoplastic hepatocytes. In conjunction with our previous immunohistochemical demonstration of a constitutively activated nuclear factor (NF)-kappaB pathway and stemness characteristics of FLHCC with limited differentiation, this cell cycle arrest elucidates the biology of FLHCC's indolent nature and relative chemoresistance.
    International journal of clinical and experimental pathology 01/2010; 3(8):792-7. · 1.78 Impact Factor
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    ABSTRACT: Certain neuroendocrine tumors (NET) metastasized to the liver can resemble hepatocellular carcinoma (HCC) in cytological needle biopsy. Experience concerning the histologic characteristics of metastatic NET resembling HCC in core needle hepatic biopsies has been scarce. The aim of this study is to describe the histological criteria in seven metastatic NET that resembled HCC in core needle hepatic biopsy. From a total of 285 needle biopsies with primary or metastasized hepatic neoplasms, seven cases were selected originally diagnosed as HCC or HCC vs. NET metastasized to the liver. Fourteen needle biopsies of hepatocellular carcinomas were also studied for comparative purposes. In all of these neoplasms the diagnosis of endocrine tumor was confirmed by immunohistochemical studies and the following information was recorded: age, sex, radiological alterations, primary site of the NET, and follow-up. The following histological data were also recorded: fibrotic stroma associated or not with the neoplastic cells, growth pattern, form of the cells, cellular size, mitotic figures, nucleomegaly, apparent nucleoli, chromatin in salt and pepper, plasmacytoid cells, intranuclear inclusions, and biliary pigment. In conclusion, these characteristics were common in metastasized neuroendocrine tumors: extensive stromal fibrosis, slight to moderate atypia, hyperchromatic nuclei, plasmacytoid cells, and thin delicate strands of fibrovascular tissue supporting larger acinar groups of net cells. HCC had a more infrequent fibrotic stroma, moderate to marked atypia, and in some biopsies biliary pigment, intranuclear inclusions, and clear cells.
    Annals of hepatology: official journal of the Mexican Association of Hepatology 9(2):186-91. · 2.19 Impact Factor
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    ABSTRACT: Fokale Veränderungen in der Leber werden seit Einführung der Abdomen-Sonographie mit einer zunehmenden Häufigkeit nachgewiesen. Diese in der Regel zufälligen Entdeckungen bedeuten für Patienten und Patientinnen einen akuten Leidensdruck, weil ganz vordergründig die potentiell bösartige Genese des Befundes in Betracht gezogen werden muss. Mit der kontroversiellen Beobachtung in den Achtzigerjahren des letzten Jahrhunderts, dass mit Einführung von Ovulationshemmern mit hohem Östrogengehalt die Inzidenz von Adenomen, deren natürlicher Verlauf sich von denen aus chirurgischen bzw. autoptischen Studien bekannten deutlich unterschied, zugenommen hatte, war die Weichenstellung zur modernen pathophysiologischen und prognostischen Differenzierung gelegt. Die moderne Histologie und Molekularbiologie sind heute in der Lage, benigne Verlaufsformen von solchen mit malignem Potential zu differenzieren.
    Wiener Medizinische Wochenschrift 03/2013; 163(5-6). DOI:10.1007/s10354-013-0177-5
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