Fibrolamellar Hepatocellular Carcinoma

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Fibrolamellär hepatocellular carcinoma (FLC) is an uncommon tumor with clinical and pathological features different from those of hepatocellular carcinoma [19]. This neoplasm occurs predominantly in young adult patients, who have no history of cirrhosis or chronic liver disease [20]. Macro-scopically, tumor size varies from 5 to 20 cm. The appearance of FLC is somewhat similar to that of focal nodular hyperplasia, with a central scar and multiple fibrous septa. Although hemorrhage is rare in FLC, necrosis and coarse calcifications have been reported in 20 to 60% of cases, especially in the central scar [20,25,39]. Most commonly, FLC is present as a solitary mass, although sometimes it may appear as bi-lobed or as a mass with small peripheral satellite lesions. Only rarely is FLC present as a diffuse multifocal mass. FLC lesions are usually intrahepatic, although sometimes pedunculated neoplasms may be found [97].

Histologically, FLC is composed of sheets of large polygonal tumor cells separated by abundant collagen bundles arranged in parallel lamellae. The tumor cells have a cytoplasm that is deeply eosinophilic and granular due to the presence of mitochondria. Sometimes FLCs contain bile [6,72,77].

The clinical presentation is variable, although patients commonly have abdominal pain, hepatomegaly, a palpable right upper quadrant abdominal mass, and cachexia [20]. Less frequently, the disease is accompanied by pain and fever, which simulates a liver abscess, gynecomastia in men, venous thrombosis, or jaundice. The gyneco-mastia is a result of the conversion of circulating androgens into estrogens by the enzyme aro-matase, which is produced by the malignant hepa-tocytes.

Venous thrombosis can occur due to invasion of the hepatic venous system or the inferior caval vein. Alternatively, it may form part of a paraneoplastic syndrome (Trousseau syndrome). Jaundice is a very rare condition, and can be caused either by invasion or compression of the biliary vessels by the tumor or by compression of the biliary vessels by enlarged lymph nodes [1,25].

The echostructure of this neoplasm is variable on US scans. Often the tumor contains both hyperand iso-echogenic components, and thus is not homogeneous. The central scar, when present, is frequently seen as a central area of hyperechogenici-ty (Fig. 25) [10,66].

On unenhanced CT images, FLC is usually seen as hypoattenuating compared to the liver and as well-defined with lobulated margins. Areas of low-density within the tumor correspond to the central scar or to necrosis and hemorrhage, while calcification may be seen in 15 to 30% of all central scars [39]. During the arterial and portal-venous phases after contrast material administration, FLC is predominantly, but heterogeneously, hyperattenuating

Heterogeneous Areas Pelvic Ultrasound
Fig. 25. Fibrolamellar carcinoma on US. Ultrasound reveals a heterogeneous hyper- to isoechoic lesion (arrows) with a hyperechoic central area (arrowhead) that corresponds to the central scar

Fig. 26a-d. Fibrolamellar carcinoma on CT. On an unenhanced CT scan (a), the neoplasm appears as a hypodense mass compared to the liver, with coarse calcification (arrowhead) and a small area of necrosis (asterisk). On arterial and portal-venous phase images after the administration of contrast material (band c, respectively), the nodule is seen as heterogeneously hyperattenuating with a hypodense central scar (arrowsin b). In the delayed phase (d) the neoplasm is hypodense and the central scar hyperdense (arrows)

Flc Hepatic Mri

Fig. 26a-d. Fibrolamellar carcinoma on CT. On an unenhanced CT scan (a), the neoplasm appears as a hypodense mass compared to the liver, with coarse calcification (arrowhead) and a small area of necrosis (asterisk). On arterial and portal-venous phase images after the administration of contrast material (band c, respectively), the nodule is seen as heterogeneously hyperattenuating with a hypodense central scar (arrowsin b). In the delayed phase (d) the neoplasm is hypodense and the central scar hyperdense (arrows)

[39]. On delayed phase images, parts of the non-necrotic portions of the tumor, which mainly comprises fibrous tissue, may increase in attenuation relative to the liver [39]. The central scar usually shows minimal enhancement on arterial and portal-venous phase images and is best seen during the delayed phase (Fig. 26). The appearance of the lesion in the arterial and portal-venous phases reflects the enhancement of the cellular and vascular components of the tumor and the presence of fibrous and necrotic components. The relative homogeneity of the tumor observed on delayed images may indicate wash-out of contrast material from the more vascular areas, together with delayed enhancement of the fibrous lamellae (Fig. 27) [39,96].

FLC is usually either hypointense or, rarely, isointense compared to the liver on T1-weighted MR images. On T2-weighted images, 90% of the lesions are hyperintense and the remaining 10% are isointense. The purely fibrous nature of the scar means that it is hypointense on both T1- and T2-weighted images (Fig. 28) [39].

FLC becomes heterogeneously hyperintense during the arterial phase after administration of gadolinium, and appears as isointense or slightly hypointense during the portal-venous and equilibrium phases (Fig. 29) [19,39]. As on CT scans, the central scar shows minimal or no enhancement on hepatic arterial and portal-venous phase images, but may sometimes show persistent enhancement on equilibrium phase images (Fig. 30).

On images acquired during the hepatobiliary phase after Gd-BOPTA or mangafodipir trisodium administration, FLC usually appears as heteroge-neously isointense or hypointense with areas of low signal intensity due to necrosis or, less frequently, hemorrhage (Fig. 31). Irregular hyperin-tense areas, if present, may be related to the presence of fibrotic components. The lack of enhancement on delayed phase images is helpful in distinguishing FLC from FNH (Fig. 32) [39].

FLC does not enhance significantly on SPIO-enhanced images. This absence of enhancement is helpful in distinguishing FLC from FNH in larger lesions, but may be less helpful in smaller lesions [66].

Flc Hepatic Mri

Fig. 27a-f. Fibrolamellar carcinoma on CT. On the precontrast CT scan (a) a large, heterogeneous lesion is demonstrated (asterisk). In the arterial phase (b) of the dynamic evaluation after contrast medium administration, markedly hyperdense intralesional vessels can be detected (arrowhead, and the lesion shows a heterogeneous density with a central hypodense scar. In the portal-venous phase (c) the mass is hypodense with a peripheral hyperdense capsule. The central scar remains hypodense in the portal-venous phase but demonstrates increased contrast density in the equilibrium phase (d). MIP reconstructions in coronal (e) and sagittal orientation (f) show the intratumoral vascularization (arrows)

Fig. 27a-f. Fibrolamellar carcinoma on CT. On the precontrast CT scan (a) a large, heterogeneous lesion is demonstrated (asterisk). In the arterial phase (b) of the dynamic evaluation after contrast medium administration, markedly hyperdense intralesional vessels can be detected (arrowhead, and the lesion shows a heterogeneous density with a central hypodense scar. In the portal-venous phase (c) the mass is hypodense with a peripheral hyperdense capsule. The central scar remains hypodense in the portal-venous phase but demonstrates increased contrast density in the equilibrium phase (d). MIP reconstructions in coronal (e) and sagittal orientation (f) show the intratumoral vascularization (arrows)

Fibrolamellar Hcc Primovist Mri
Fig. 28a, b. Fibrolamellar carcinoma. Unenhanced Tl-weighted (a) and T2-weighted (b) images reveal a neoplasm that is hypointense and hyperintense compared to the normal parenchyma, respectively. On both images a hypointense central scar (arrowhead is evident
Fibrolamellar Hepatocellular CarcinomaFibrolamellar Hcc Liver Mri

Fig. 29a-g. Fibrolamellar carcinoma. On the unenhanced T2-weighted image (a), a large hyperintense lesion can be noted in the dome of the liver. On the corresponding unenhanced T1-weighted image (b) this lesion appears as heterogeneously hy-pointense. Dynamic imaging after the bolus injection of Gd-BOPTA reveals hyper-vascularization of the periphery of the lesion during the initial arterial phase (c) followed by filling-in in the subsequent portal-venous phase (d). During the equilibrium phase (e) a hypointense appearance is evident even in peripheral areas. In contrast to focal nodular hyperplasia (FNH), the central scar of this lesion shows no enhancement in the equilibrium phase. On T1-weighted images acquired during the hepatobiliary phase 1h after the injection of Gd-BOPTA (f, g) the lesion shows peripheral wash-out and a hypointense central scar. Non-specific enhancement due to diffusion of the contrast agent into different parts of the lesion can be noted. Additionally, peripheral satellite nodules (arrows in g) can be seen in images acquired more caudally (g). In contrast to FNH, the lesion shows a higher signal intensity on unenhanced T2-weighted images and more pronounced hypointensity on unen-hanced Tl-weighted images. In the equilibrium phase no enhancement of the central scar can be noted and no uptake of Gd-BOPTA by the lesion is apparent on hepatobiliary phase images. This behavior is consistent with the presence of non-functioning hepatocytes and hence malignancy

Right Heart Ventricle MriFibrolamellar Carcinoma Liver

Fig. 30a-e. Fibrolamellar carcinoma. On the unenhanced T2-weighted image (a), a large heterogeneously hyperintense lesion can be seen (arrows). On the corresponding unenhanced VIBE image (b) the lesion appears heterogeneously hy-pointense. Dynamic evaluation after the bolus injection of Gd-BOPTA reveals heterogeneous hypervascularization of the lesion during the arterial phase (c) followed by contrast agent wash-out in the subsequent portal-venous (d) and equilibrium (e) phases

Fig. 30a-e. Fibrolamellar carcinoma. On the unenhanced T2-weighted image (a), a large heterogeneously hyperintense lesion can be seen (arrows). On the corresponding unenhanced VIBE image (b) the lesion appears heterogeneously hy-pointense. Dynamic evaluation after the bolus injection of Gd-BOPTA reveals heterogeneous hypervascularization of the lesion during the arterial phase (c) followed by contrast agent wash-out in the subsequent portal-venous (d) and equilibrium (e) phases

Arterial Phase Liver Mri

Fig. 31a, b. Fibrolamellar carcinoma after contrast agents. On the hepatobiliary phase after the injection of Gd-BOPTA (a) the lesion appears hypointense with a hyperintense central scar (arrowheads). In a similar way, the mass appears hypointense in the hepatobiliary phase after injection of Mn-DPDP with a markedly hypointense central area corresponding to the scar (b)

Fig. 31a, b. Fibrolamellar carcinoma after contrast agents. On the hepatobiliary phase after the injection of Gd-BOPTA (a) the lesion appears hypointense with a hyperintense central scar (arrowheads). In a similar way, the mass appears hypointense in the hepatobiliary phase after injection of Mn-DPDP with a markedly hypointense central area corresponding to the scar (b)

Fig. 32a-d. Fibrolamellar carcinoma after Gd-BOPTA. Fibrolamellar carcinomas do not show significant enhancement on delayed hepatobiliary phase images after the bolus administration of Gd-BOPTA (a and b, respectively). Conversely, focal nodular hyperplasia generally appears hyperintense on delayed liver-specific phase images after Gd-BOPTA due to the accumulation of contrast agent within the nodule and impaired biliary excretion (c and d, respectively)

Fibrolamellar Hepatocellular Carcinoma

Fig. 32a-d. Fibrolamellar carcinoma after Gd-BOPTA. Fibrolamellar carcinomas do not show significant enhancement on delayed hepatobiliary phase images after the bolus administration of Gd-BOPTA (a and b, respectively). Conversely, focal nodular hyperplasia generally appears hyperintense on delayed liver-specific phase images after Gd-BOPTA due to the accumulation of contrast agent within the nodule and impaired biliary excretion (c and d, respectively)

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