eMedicine Specialties > Radiology > Gastrointestinal
Hepatocellular Carcinoma, Fibrolamellar: Imaging
Updated: Aug 22, 2007
Radiography
Findings
Plain radiographs of the abdomen may demonstrate nonspecific secondary findings related to hepatomegaly or to the abdominal mass in patients with fibrolamellar carcinoma. Findings may include an enlarged liver shadow and/or displacement of adjacent, gas-filled bowel loops. Calcifications in the tumor also may be visible on plain abdominal images.
Degree of Confidence
Plain radiographic findings in patients with fibrolamellar carcinoma are nonsensitive and nonspecific. These findings are not helpful in the detection or characterization of the tumors.
False Positives/Negatives
Any cause of space-occupying abdominal masses or hepatomegaly can produce plain radiographic findings similar to those of fibrolamellar carcinoma, thereby causing a false-positive finding. Changes associated with fibrolamellar carcinoma may not produce any abnormality on plain radiographs, thereby causing a false-negative result.
Computed Tomography
Findings
Abdominal CT scanning is the preferred imaging method for the diagnosis, staging, and follow-up surveillance of fibrolamellar carcinoma. CT scanning has high sensitivity in the detection of intrahepatic tumors and regional lymph node metastases. Current-generation CT scanners provide the ability to scan the liver without enhancement and, during the arterial and portal venous phases, after the intravenous administration of iodinated contrast material.
On nonenhanced scans, the primary fibrolamellar tumor typically appears as a large, solitary, hypoattenuating mass with well-circumscribed and lobulated margins. During the arterial-enhancing phase, the tumor is heterogeneously enhancing and becomes generally hyperattenuating with respect to the relatively less strongly enhancing surrounding liver. During the portal and delayed phases, the tumor remains enhancing and becomes more homogeneous in appearance, with its density more closely matching that of the liver as equilibrium is achieved.
Central scars are present in 50-70% of fibrolamellar carcinomas and appear on CT scans as a central stellate hypoattenuating and hypoenhancing region in the mass. The scars may not be enhancing at all, or they may show mild enhancement on delayed-enhanced scans. Calcifications occur in 35-60% of fibrolamellar tumors, and they can be best identified as hyperattenuating foci on nonenhanced CT scans.
Metastatic lymphadenopathy is present at the time of initial diagnosis of fibrolamellar carcinoma in 50-70% of patients, and the lymph nodes are frequent sites for recurrent disease after surgical resection of primary lesions. Lymph node metastases are found most often in the porta hepatis, and they can have a CT scan appearance similar to that of intrahepatic lesions.
Other distant metastases, such as those to the lungs, peritoneum, or more distant lymph nodes, also can be detected by using CT scanning.
Degree of Confidence
Abdominal CT scanning is sensitive in detecting the primary intrahepatic tumors of fibrolamellar carcinoma and of regional lymph node metastases. When found in the appropriate clinical setting of a young patient without cirrhosis, the characteristic CT scan appearance of a large, lobulated, heterogeneously enhancing tumor with a central scar and calcification, often with local lymph node metastases, can provide a confident diagnosis of fibrolamellar carcinoma. Because most patients are treated with surgical resection, percutaneous biopsy may be useful in the preoperative confirmation of the diagnosis.
False Positives/Negatives
In an atypical clinical setting, fibrolamellar carcinomas may be confused with hepatocellular adenomas or HCC, which can have a similar appearance. FNH can appear similar to fibrolamellar tumors, but its enhancement tends to be more homogeneous. Also, FNH has an enhancing scar, and it does not metastasize.
Magnetic Resonance Imaging
Findings
Fibrolamellar carcinoma is typically identified on magnetic resonance images as a large, well-defined, lobulated mass. On T1-weighted images, the tumors tend to be mostly homogeneous and hypointense relative to the liver. A minority of tumors may be heterogeneous and isointense. On T2-weighted images, the tumors are commonly heterogeneous and, most often, hyperintense with respect to the liver.3
The central scar usually appears hypointense on all images obtained with all sequences. The appearance of the scar on magnetic resonance images can be useful in differentiating fibrolamellar carcinomas from FNH, because scars in FNH tend to be hyperintense on T2-weighted images.
Fibrolamellar carcinomas typically do not contain intracellular fat; therefore, the presence of fat on fat-saturated, in-phase, and out-of-phase magnetic resonance images suggests HCC or an adenoma.
On gadolinium-enhanced magnetic resonance images, the enhancement patterns seen in fibrolamellar carcinomas are similar to those seen on contrast-enhanced CT scans. Early heterogeneous enhancement occurs during the arterial phase and progresses to more homogeneous enhancement during delayed phases. The central scar does not enhance during the arterial phase, but it may demonstrate mild enhancement in the later portal or equilibrium phases.
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or magnetic resonance angiography (MRA) scans. As of late December 2006, the Food and Drug Administration had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on thewhites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
Degree of Confidence
MRI is sensitive in the detection of intrahepatic masses of fibrolamellar carcinoma, and MRI may be slightly more sensitive than CT scanning in depicting multiple recurrent intrahepatic lesions. Although metastatic lymphadenopathy can be seen on magnetic resonance images, CT scanning is probably more useful for detecting distant metastases, particularly in the lungs. CT scanning is also relatively less expensive than MRI; therefore, CT scanning is most appropriate for initial imaging and follow-up in most patients.
MRI is most useful in the characterization of indeterminate masses seen on CT scans. Often, MRI features of tumors can help to confirm a suggested diagnosis of fibrolamellar carcinoma, or they can exclude the possibility of other entities such, as FNH or HCC.
False Positives/Negatives
As with CT scanning, MRI is not entirely specific for fibrolamellar carcinoma, and other liver lesions (eg, adenoma, HCC, FNH) can occasionally mimic the appearance of fibrolamellar carcinoma. For instance, the hyperintense scar that is usually associated with FNH has been reported in at least 1 patient with fibrolamellar carcinoma.
Ultrasonography
Findings
Abdominal ultrasonograms are often obtained for the initial evaluation of patients with abdominal pain; therefore, ultrasonography may be the first imaging study available in patients with fibrolamellar carcinoma. On ultrasonograms, the primary tumor can be seen as a solitary, well-defined hepatic mass with a heterogeneous echotexture. The tumor's scar may be seen as a central, hyperechoic structure, and calcification may be represented by an echogenic focus with shadowing.
Degree of Confidence
Because the primary intrahepatic tumors of fibrolamellar carcinoma are often large, the likelihood of detecting the mass with ultrasonography is high. However, ultrasonography is less sensitive than CT scanning in depicting tumoral characteristics, such as scars and calcifications, which aid in the differentiation of fibrolamellar carcinoma from other intrahepatic tumors. Ultrasonography is less accurate than CT scanning in staging extrahepatic disease. After the initial detection of the intrahepatic mass with ultrasonography, further evaluation with CT scanning or MRI is usually necessary for the definitive diagnosis and staging of fibrolamellar carcinoma.
False Positives/Negatives
Although, in a young patient, a large intrahepatic mass with a central scar is suggestive of fibrolamellar carcinoma, other entities (eg, FNH, hepatocellular adenoma, possibly giant cavernous hemangioma) may have a similar appearance on ultrasonograms and result in false-positive findings.
False-negative ultrasonographic findings may occur in patients with obesity, underlying cirrhosis or fatty infiltration (which is not typical in patients with fibrolamellar carcinoma), or a body habitus that may obscure good visualization of the liver during ultrasonography.
Nuclear Imaging
Findings
Radionuclide imaging studies are not useful in the detection of fibrolamellar carcinoma. However, in some patients, these studies may be useful in the differential diagnosis of the tumors.
On sulfur-colloid liver-spleen scans, fibrolamellar carcinomas appear as photopenic defects in the liver; the masses demonstrate decreased activity. On scans obtained with red blood cells labeled with technetium-99m, fibrolamellar carcinomas typically demonstrate increased activity on early arterial-phase images. This finding is followed by decreased activity with respect to the surrounding liver on delayed-phase images.
Degree of Confidence
In difficult or equivocal cases, radionuclide sulfur-colloid images or images obtained with labeled red blood cells may provide information that increases the level of confidence in the diagnosis of fibrolamellar carcinoma. However, these studies are not useful in detecting or staging fibrolamellar carcinoma.
False Positives/Negatives
Sulfur-colloid liver-spleen scans can demonstrate photopenic lesions in the liver with many entities, including benign and malignant liver tumors, cysts, and abscesses; therefore, false-positive results are possible. False-negative sulfur-colloid scans may occur when diffuse hepatocellular dysfunction, such as cirrhosis, results in poor tracer uptake in a noncancerous liver.
Other liver tumors may have uptake patterns on labeled red blood cell scans that are similar to the uptake pattern of fibrolamellar carcinoma; therefore, false-positive findings can result. The tumors may demonstrate normal activity levels, resulting in a false-negative finding.
Angiography
Findings
Angiography is usually not beneficial in the detection, staging, or evaluation of fibrolamellar carcinoma. If obtained, angiograms demonstrate the primary tumor as a hypervascular mass with multiple feeding arteries, a dense tumor blush, and an avascular central scar.
More on Hepatocellular Carcinoma, Fibrolamellar |
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 Imaging: Hepatocellular Carcinoma, Fibrolamellar |
| Follow-up: Hepatocellular Carcinoma, Fibrolamellar |
| Multimedia: Hepatocellular Carcinoma, Fibrolamellar |
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Further Reading
Keywords
HCC, fibrolamellar carcinoma, fibrolamellar hepatoma, oncocytic hepatocellular carcinoma
