The fibrolamellar variant of hepatocellular carcinoma. Its association with focal nodular hyperplasia

Abstract

A case of fibrolamellar hepatocellular carcinoma (FL-HCC) associated with adjacent focal nodular hyperplasia (FNH) is described. These two regions were adjacent but distinct, both on gross and microscopic examination. Currently, it is unclear whether FL-HCC rarely arises in preexisting FNH, or whether FNH is a typical response to this vascular variant of hepatocellular carcinoma (HCC). The FNH region, which is peripheral, may be biopsied to exclude the underlying carcinoma, and thus lead to inadequate therapy. Previous reports of this association are reviewed. importance of this observation in terms of patient management and proposed pathogenesis is discussed. Case ReportA 19-year-old woman with a 3-year history of oral contraceptive use (type unknown) was admitted for liver mass evaluation. One and one half months before she was admitted, sharp, episodic, left-sided chest pain developed. Hepatomegaly without splenomegaly was detected at evaluation. A computed tomography (CT) scan showed a hepatic mass. She was transferred to the Hospital of the University of Pennsylvania for further examination. The results of the physical examination were normal except for an 1 I-cm liver (by percussion), which was nontender but palpable 5.0 cm below the right costal margin. Complete blood count and serum electrolytes were normal, as were the alanine and aspartate aminotransferases and gamma glutamyl transpeptidase. Serum alkaline phosphatase was minimally elevated at 256 IU/L (compressed normal hepatic parenchyma < 140 IU/L). Serum alpha-fetoprotein and carcinoembryonic antigen were less than 10 ng/ml and 3 ng/ml, respectively. A nonenhanced and contrast-enhanced abdominal CT scan showed a large, relatively hypodense mass in the right hepatic lobe, with an enhancing central septate region. Selective right hepatic arteriography demonstrated a large hypervascular mass in the inferior portion of the right lobe, with a peripheral arterial supply radially penetrating into the center of the lesion (Figure 1). The parenchymal phase showed a dense capillary blush with a stellate septate pattern and central lucency compatible with a central scar. Vascular encasement. portal vein thrombosis, or arteriovenous shunting were not detected. The lesion was considered consistent with FNH.The patient was operated on and the anterior segment of the right hepatic lobe was removed and sent for pathologic con- 3049

Hepatocellular carcinoma associated with focal nodular hyperplasia

“…The brown portion was a classical HCC. The associations of HCC with adjacent FNH were rarely described [1,11,12]. In contrast to these reported cases, the HCC component in our case was not a fibrolamellar variant but a classical type.…”

“…It has been recently shown to be a clonal proliferative disease using HUMARA (methylation pattern of the polymorphic X-chromosome-linked androgen receptor gene) analysis [2]. Its associations with fibrolamellar hepatocellular carcinoma (HCC) were also reported [1,11,12]. Although some reports indicated an association between FNH and HCC, most authors did not consider a pathogenetic correlation between them.…”

Hepatic focal nodular hyperplasia in children previously treated for a solid tumor

“…19 -21 In contrast with FNH, fibrolamellar carcinoma may demonstrate a central scar with low signal intensity on T2-weighted images. 27,28 In patients with a history of malignancy, a solitary hypervascular lesion may simulate a metastasis; however, most metastases do not exhibit a central scar. 29 Liver cell adenoma is a benign tumor that is devoid of a central scar and for which surgical resection is justified because of the high incidence of bleeding complications and possible malignant degeneration.…”

Fibrolamellar Carcinoma of the Liver: Composition of the Extracellular Matrix and Expression of Cell–Matrix and Cell–Cell Adhesion Molecules

“…48 The number of cases included in the present study is too low to allow reliable clinicopathoof focal nodular hyperplasia, of which FLC has been sometimes considered the malignant counterpart. 31,32 In particu-logical correlations. Further studies are needed to evaluate whether, as previously suggested, 48,49 the phenotypic heterolar, the large amounts of tenascin present in the stroma of FLC are absent from the central scars characteristic of focal geneity of FLC indicates the existence of distinct subgroups in this tumor variant, and whether it may be of prognostic nodular hyperplasia, as shown by a previous study from our laboratory 25 and as confirmed in the present study.…”