Fibrolamellar Carcinoma

 Uncommon malignant hepatocellular tumor



• Also known as fibrolamellar hepatocellular carcinoma (HCC)
image Distinct clinical, histopathologic, and imaging features differentiate it from conventional HCC




IMAGING




• Heterogeneously enhancing, large, lobulated mass with hypointense central scar and radial septa
image Calcification and necrosis are common (> 50%)

image Nodal metastases (> 50%)

image Vary from 5-20 cm (mean: 13 cm)

image “Satellite” nodules are often present

• Slow-growing tumor that usually arises in normal (noncirrhotic) liver

• Better prognosis than conventional HCC but still locally invasive and frequently metastatic


TOP DIFFERENTIAL DIAGNOSES




• Focal nodular hyperplasia (FNH)

• Conventional HCC

• Hepatic cavernous hemangioma

• Peripheral cholangiocarcinoma


DIAGNOSTIC CHECKLIST




• FLC simulates FNH due to presence of central scar in both tumors
image FLC: Bigger, more heterogeneous mass frequently with calcified central/eccentric scar and features of malignancy (vessel &/or biliary obstruction, nodal invasion, and lung metastases)

image Scar on T2WI: Hypointense in FLC,  hyperintense in FNH

• Large, heterogeneous, hypervascular tumor in young adult

image
(Left) Axial graphic shows a large, heterogeneous, hypervascular mass with a central scar and porta hepatis lymphadenopathy image.


image
(Right) Axial CECT in a 15-year-old boy shows a large, heterogeneous, hypervascular mass image with a large, calcified central scar image and cardiophrenic lymphadenopathy image. In a young person, these findings are essentially diagnostic of fibrolamellar carcinoma (FLC)

image
(Left) Axial CECT of a 22-year-old man with abdominal discomfort and a palpable mass shows a large, heterogeneous, lobulated mass image with a large central scar containing foci of calcification image. Scar calcification is very rare in focal nodular hyperplasia (FNH), by comparison.


image
(Right) Gross pathology of the same patient’s resected tumor shows a well-demarcated, lobulated, heterogeneous tumor with bile staining and central or eccentric fibrous scars image, typical features of FLC.


TERMINOLOGY


Abbreviations




• Fibrolamellar carcinoma (FLC) of liver


Synonyms




• Fibrolamellar hepatocellular carcinoma


Definitions




• Uncommon malignant hepatocellular tumor
image Distinct clinical, histopathologic, and imaging features differentiate it from conventional hepatocellular carcinoma (HCC)


IMAGING


General Features




• Best diagnostic clue
image Heterogeneously enhancing, large, lobulated mass with hypointense central scar and radial septa

• Location
image Intrahepatic (80%)

image Pedunculated (20%)

• Size
image 5-20 cm (mean: 13 cm)

• Key concepts
image Slow-growing tumor that usually arises in normal (noncirrhotic) liver
– May occur with underlying cirrhosis (< 5% of cases)

image “Satellite” nodules are often present

image Characteristic microscopic pattern
– Eosinophilic malignant hepatocytes containing prominent nuclei

image Absence of pathologic markers (e.g., inclusions of α-fetoprotein bodies) that are present in conventional HCC

image Better prognosis than conventional HCC but still locally invasive and frequently metastatic


CT Findings




• NECT
image Mass
– Well-defined contour

– Hypoattenuating and heterogeneous

image Central scar and septa: Markedly hypodense

image Calcification and necrosis are common (> 50%)

image Hemorrhage is rarely seen

• CECT
image Arterial phase
– Mass: Heterogeneous and hyperattenuating (80%)

image Portal phase
– Mass: Iso-/hypoattenuating

image Delayed phase (10 min)
– Mass: Isodense

– Scar and septa: Hyperdense

image Malignant features
– Biliary and vessel invasion

– Nodal metastases (> 50%)
image Porta hepatis and cardiophrenic nodes

– Lung metastases


MR Findings




• T1WI
image Mass: Heterogeneous and slightly hypointense

image Scar and septa: Hypointense

• T2WI
image Mass: Heterogeneous and hyperintense

image Scar and septa: Hypointense

• T1WI C+
image Arterial and portal phases
– Intense heterogeneous enhancement of mass, not scar

image Delayed phase
– Mass: More homogeneous enhancement

– Scar and septa: Delayed partial enhancement


Ultrasonographic Findings




• Grayscale ultrasound
image Mass
– Large, solitary, well defined, and lobulated

– Variable echotexture

image Central scar: Hyperechoic


Angiographic Findings




• Conventional
image Mass
– Hypervascular (neovascularity)

– Enlarged feeding arteries

– Dense tumor blush

– No arterial-venous or arterial-portal shunting

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Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Fibrolamellar Carcinoma

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