eMedicine Specialties > Radiology > Gastrointestinal

Hepatocellular Carcinoma, Fibrolamellar

Updated: Aug 22, 2007

Introduction

Background

Fibrolamellar hepatocellular carcinoma, or fibrolamellar carcinoma, is an uncommon malignant neoplasm of the liver. Fibrolamellar carcinoma has distinctive clinical, histologic, and radiographic features that distinguish it from the relatively more common hepatocellular carcinoma (HCC). Fibrolamellar carcinoma occurs in a younger population than does HCC, and it is typically not associated with underlying liver disease or elevated serum levels of alpha-fetoprotein tumor markers.¹ Also, fibrolamellar carcinoma may have a slightly better prognosis than HCC.

Perhaps because of the younger ages of the patients and the lack of coexisting cirrhosis, patients with fibrolamellar carcinoma are often treated aggressively. Resection of large tumor masses, of metastatic disease, and even of recurrent disease can extend patient survival. Radiographic evaluation of patients with fibrolamellar carcinoma may be used for the initial diagnosis of the tumor, for the preoperative staging of the disease, and for follow-up surveillance to detect recurrent or metastatic disease.

Pathophysiology

Gross findings

Fibrolamellar carcinoma most commonly presents as a large, solitary intrahepatic mass with well-defined margins and a lobulated contour. The tumors are typically large at the time of diagnosis, with a mean diameter of 10-20 cm. Regional lymph node metastases are found in 50-70% of patients at the time of initial diagnosis. Portal or hepatic venous invasion is not typical. Grossly, the primary tumor appears as a well-demarcated, lobular, bile-stained, white or tan mass with a central stellate scar or fibrotic bands. Hemorrhage and necrosis are uncommon.

Central calcification is present in 35-60% of tumors and thus can be a useful diagnostic feature. However, fibrolamellar carcinomas are not the only liver tumors in which calcifications appear; calcifications may also be found in a minority of HCCs, as well as in some metastatic tumors, such as those arising from mucinous colon or ovarian carcinomas.

Microscopic findings

Microscopically, fibrolamellar carcinomas have a characteristic pattern of nests, sheets, or cords of malignant cells, which are separated by lamellar bands of dense, hypocellular collagen connective tissue. The fibrotic connective tissue coalesces into the central scar. The malignant cells are usually well-differentiated polygonal cells containing granular cytoplasm, large nuclei, and prominent nucleoli.

Metastatic lesions appear to be histopathologically similar to the primary tumors.

The internal architecture of fibrolamellar carcinomas may be heterogeneous, and foci of focal nodular hyperplasia (FNH) may occur in the liver, adjacent to the tumors. For accurate diagnosis at percutaneous biopsy, the acquisition of multiple core specimens is recommended to avoid misdiagnosis resulting from sampling error.

Staging

Tumors may be graded histologically according to the Broders method, as follows²:

In grade 1 tumors, 75-100% of the tumor cells are well differentiated.
In grade 2 tumors, 50-75% of the tumor cells are well differentiated.
In grade 3 tumors, 25-50% of the tumor cells are well differentiated.
In grade 4 tumors, 0-25% of the tumor cells are well differentiated.

Frequency

United States

HCC is the most common primary malignancy of the liver and accounts for approximately 2-3% of all cancers in the United States. Fibrolamellar carcinoma accounts for fewer than 10% of all cases of HCC, but it accounts for approximately 35% of all cases of HCC in patients younger than 50 years who do not also have cirrhosis.

International

The more common form of HCC has a greater prevalence in Asia than in it does in the United States, accounting for as many as 40% of all cancer cases in endemic areas. However, the prevalence of fibrolamellar carcinoma in Asia is lower than it is in the United States.

Mortality/Morbidity

In patients with fibrolamellar carcinoma, the reported 5-year survival rates are approximately 25-30% if all patients are included. However, the rates increase to as high as 63% in patients in whom tumors are resected.
Long-term survival as long as 14 years has been reported.
Although the postoperative recurrence of fibrolamellar carcinoma is high, ranging from 42-100% in reported series, aggressive surgical management, including the resection of recurrent and metastatic disease, has been reported to improve survival rates and disease-free intervals.

Race

No racial predilection has been reported in fibrolamellar carcinoma.

Sex

No clear sex predilection has been reported in fibrolamellar carcinoma.

Age

Fibrolamellar carcinoma occurs primarily in young adults. Reported series indicate a patient age range of 5-69 years, with a mean age of 23 years at the time of initial diagnosis.

Anatomy

Fibrolamellar carcinoma may occur anywhere in the liver. Anatomically, the liver is composed of right, left, and caudate lobes. It can be further divided into 8 surgical subsegments based on the locations of the major portal and hepatic venous structures. Attention to the location and extent of fibrolamellar tumors with respect to the surgical anatomy is important because tumor resection is recommended in most patients.

Metastatic tumoral involvement is most common in the regional lymph nodes of the porta hepatis or celiac axis. Distant metastases to the lungs, paraspinal tissues, and supraclavicular lymph nodes have been reported.

Presentation

Presentation

Patients with fibrolamellar carcinoma may present with abdominal pain, a palpable abdominal mass, hepatomegaly, or cachexia. Jaundice is not common. Serum alpha-fetoprotein levels are within the reference range or mildly elevated. Liver function test results may be elevated. Patients typically do not have hepatitis or cirrhosis. Computed tomography (CT) scan findings usually suggest the diagnosis.

Staging

Fibrolamellar carcinoma is usually advanced at the time of initial diagnosis. Primary tumors are large, with a reported mean diameter of 10-20 cm. Extrahepatic disease, most often in regional lymph nodes, is present in as many as 80% of patients at the time of initial diagnosis. Portal or hepatic venous invasion may occur, but it is not common. Diffuse intraperitoneal carcinomatosis has been reported. Distant metastases are typically not found at the time of presentation.

Recurrence and follow-up

Fibrolamellar carcinoma is an aggressive tumor that in most patients progresses to recurrent liver masses and metastatic lymph node metastases. Recurrent lesions often develop 6-18 months after attempted curative resection and may progress rapidly; therefore, follow-up imaging is recommended at 2- to 4-month intervals for at least 12-18 months after resection of the primary tumor. The early detection of metastatic disease is important because surgical resection of metastases improves patient survival rates.

Preferred Examination

Abdominal CT scanning is the preferred examination for the detection, diagnosis, staging, and postoperative follow-up evaluation of fibrolamellar carcinoma.

Magnetic resonance imaging (MRI) of the liver can be useful in detecting and characterizing primary tumors, and MRI may be slightly more sensitive than CT scanning in detecting multiple intrahepatic recurrent lesions. However, MRI is less sensitive than CT scanning in the detection of extrahepatic disease.

Ultrasonography has been used in the surveillance of the progression of known intrahepatic lesions. Radionuclide sulfur-colloid scans or scans obtained with labeled red blood cells are occasionally useful in the differentiation of fibrolamellar carcinomas from other types of hepatic tumors. Percutaneous biopsy with CT scanning or ultrasonographic guidance may be necessary for a definitive preoperative diagnosis of fibrolamellar carcinoma.

Limitations of Techniques

For the best evaluation of the liver and liver tumors with CT scanning, the intravenous administration of iodinated contrast material is necessary. Therefore, the use of CT scanning is limited in patients who cannot have iodinated contrast material because of an allergy or renal insufficiency. In these patients, MRI is preferred. In addition, CT scanning uses ionizing radiation; therefore, it can be relatively contraindicated in children or in women of childbearing age, in whom radiation doses should be limited as much as possible.

The imaging time with MRI examinations tends to be longer than with CT scanning or ultrasonography. In patients who are unwilling or unable to remain still during the imaging period, poor-quality images can result because of motion artifact; these artifacts can limit the usefulness of the MRI examination. Some patients may become claustrophobic while they are in the MRI machine; patients may require sedation, or they may not be able to complete the examination. Because of the high magnetic field strength, MRI is contraindicated in patients with a cardiac pacemaker or some internal metallic object, such as an aneurysm clip or metal shrapnel or filings in a critical location.

Ultrasonography is more operator dependent than is CT scanning or MRI, and poor technique in scanning can limit its diagnostic usefulness. Ultrasonography may be limited by the patient's anatomy. Hepatic imaging may be difficult in obese patients or in patients whose liver is located high under the ribs. Overlying bowel gas may obscure portions of the liver in some patients.

Differential Diagnoses

Focal Nodular Hyperplasia
Hepatic Adenoma
Hepatocellular Carcinoma

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.

Intervention

A percutaneous biopsy of a fibrolamellar carcinoma may be obtained for a definitive preoperative diagnosis. A percutaneous biopsy specimen is usually obtained by using either CT scanning or ultrasonographic guidance. Routine image-guided biopsy techniques are used. Fibrolamellar carcinomas are often pathologically heterogeneous, and they may contain areas of HCC or neuroendocrine differentiation. Nodular hyperplastic changes in the liver, adjacent to the tumor, may mimic FNH. Therefore, in patients with suggested fibrolamellar carcinoma, the acquisition of multiple core-needle biopsy samples is recommended to avoid misdiagnosis from sampling error. Potential complications of image-guided biopsy of fibrolamellar carcinoma are uncommon and include hemorrhage and infection.

Aggressive surgical treatment is used in patients with fibrolamellar carcinoma. The initial treatment usually involves resection of the primary tumor or liver transplantation, with en bloc resection of metastatic lymphadenopathy. Despite aggressive initial therapy, tumor recurrence within 2-12 months of resection with intent to cure is typical. Tumor recurrence almost always involves the liver, and it may be unifocal or multifocal. Intrahepatic recurrence is not necessarily located at the surgical margins. In addition, recurrent regional lymphadenopathy is often present. Distant metastases to lung or other remote sites may occur late in the course of the disease.

Surgical resection of recurrent and metastatic tumors has been shown to improve survival and is recommended. Patients with unresectable lesions can be treated with chemotherapy. Radiation therapy has not been reported to be useful.

Multimedia

Media file 1: Contrast-enhanced computed tomography (CT) scan of fibrolamellar carcinoma demonstrates a large, heterogeneously enhancing mass in the right lobe of the liver, with a hypoattenuating central scar and punctate, central calcification.

Media file 2: Photograph of a resected gross pathologic specimen of fibrolamellar carcinoma shows a lobulated, tan-colored mass with a stellate, central scar (same patient as in Image 1).

Media file 3: Computed tomography (CT) scan in a patient with fibrolamellar carcinoma shows metastatic lymphadenopathy in the porta hepatis–gastrohepatic region.

Media file 4: T1-weighted magnetic resonance image of the liver in a patient with fibrolamellar carcinoma shows a lobulated and well-defined mass with a relatively homogeneous, hypointense appearance.

Media file 5: T2-weighted magnetic resonance image (same patient as in Image 4) shows a fibrolamellar carcinoma with increased signal intensity relative to that of the surrounding liver.

Media file 6: Ultrasonogram shows a heterogeneous echotexture mass in the liver that corresponds to a fibrolamellar carcinoma.

Media file 7: Digital subtraction arteriogram (obtained by injection of the celiac axis) demonstrates fibrolamellar carcinoma; the carcinoma appears as an area of hypervascularity and tumor blush in the inferior aspect of the liver.

References

Titelbaum DS, Burke DR, Meranze SG, et al. Fibrolamellar hepatocellular carcinoma: pitfalls in nonoperative diagnosis. Radiology. Apr 1988;167(1):25-30. [Medline]. [Full Text].
Broders AC. The grading of carcinoma. Minn Med. 1925;8:726-30.
McLarney JK, Rucker PT, Bender GN, et al. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics. Mar-Apr 1999;19(2):453-71. [Medline]. [Full Text].
Bedi DG, Kumar R, Morettin LB, et al. Fibrolamellar carcinoma of the liver: CT, ultrasound and angiography. Case report. Eur J Radiol. May 1988;8(2):109-12. [Medline].
Berman MM, Libbey NP, Foster JH. Hepatocellular carcinoma. Polygonal cell type with fibrous stroma--an atypical variant with a favorable prognosis. Cancer. Sep 15 1980;46(6):1448-55. [Medline].
Brandt DJ, Johnson CD, Stephens DH, et al. Imaging of fibrolamellar hepatocellular carcinoma. AJR Am J Roentgenol. Aug 1988;151(2):295-9. [Medline].
Craig JR, Peters RL, Edmondson HA, et al. Fibrolamellar carcinoma of the liver: a tumor of adolescents and young adults with distinctive clinico-pathologic features. Cancer. Jul 15 1980;46(2):372-9. [Medline].
Farhi DC, Shikes RH, Murari PJ, et al. Hepatocellular carcinoma in young people. Cancer. Oct 15 1983;52(8):1516-25. [Medline].
Farhi DC, Shikes RH, Silverberg SG. Ultrastructure of fibrolamellar oncocytic hepatoma. Cancer. Aug 15 1982;50(4):702-9. [Medline].
Francis IR, Agha FP, Thompson NW, et al. Fibrolamellar hepatocarcinoma: clinical, radiologic, and pathologic features. Gastrointest Radiol. 1986;11(1):67-72. [Medline].
Friedman AC, Lichtenstein JE, Goodman Z, et al. Fibrolamellar hepatocellular carcinoma. Radiology. Dec 1985;157(3):583-7. [Medline]. [Full Text].
Ichikawa T, Federle MP, Grazioli L, et al. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology. Nov 1999;213(2):352-61. [Medline]. [Full Text].
Ichikawa T, Federle MP, Grazioli L, et al. Fibrolamellar hepatocellular carcinoma: pre- and posttherapy evaluation with CT and MR imaging. Radiology. Oct 2000;217(1):145-51. [Medline]. [Full Text].
Joishy SK, Balasegaram M. Hepatic resection for malignant tumors of the liver: essentials for a unified surgical approach. Am J Surg. Mar 1980;139(3):360-9. [Medline].
Lack EE, Neave C, Vawter GF. Hepatocellular carcinoma. Review of 32 cases in childhood and adolescence. Cancer. Oct 15 1983;52(8):1510-5. [Medline].
Moreno-Luna LE, Arrieta O, García-Leiva J, et al. Clinical and pathologic factors associated with survival in young adult patients with fibrolamellar hepatocarcinoma. BMC Cancer. 2005;5:142. [Medline]. [Full Text].
Mukai JK, Stack CM, Turner DA, et al. Imaging of surgically relevant hepatic vascular and segmental anatomy. Part 2. Extent and resectability of hepatic neoplasms. AJR Am J Roentgenol. Aug 1987;149(2):293-7. [Medline].
Nagorney DM, Adson MA, Weiland LH, et al. Fibrolamellar hepatoma. Am J Surg. Jan 1985;149(1):113-9. [Medline].
Soreide O, Czerniak A, Bradpiece H, et al. Characteristics of fibrolamellar hepatocellular carcinoma. A study of nine cases and a review of the literature. Am J Surg. Apr 1986;151(4):518-23. [Medline].
Soyer P, Roche A, Levesque M, et al. CT of fibrolamellar hepatocellular carcinoma. J Comput Assist Tomogr. Jul-Aug 1991;15(4):533-8. [Medline].
Stevens WR, Johnson CD, Stephens DH, et al. Fibrolamellar hepatocellular carcinoma: stage at presentation and results of aggressive surgical management. AJR Am J Roentgenol. May 1995;164(5):1153-8. [Medline].
Stipa F, Yoon SS, Liau KH, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. Mar 15 2006;106(6):1331-8. [Medline].
Vecchio FM. Fibrolamellar carcinoma of the liver: a distinct entity within the hepatocellular tumors. A review. Appl Pathol. 1988;6(2):139-48. [Medline].
Wetzel WJ, Costin JL, Petrino RL. Fibrolamellar carcinoma: distinctive clinical and morphologic variant of hepatoma. South Med J. Jun 1983;76(6):796-8. [Medline].
Wong LK, Link DP, Frey CF, et al. Fibrolamellar hepatocarcinoma: radiology, management, and pathology. AJR Am J Roentgenol. Jul 1982;139(1):172-5. [Medline].
Wood WJ, Rawlings M, Evans H, et al. Hepatocellular carcinoma: importance of histologic classification as a prognostic factor. Am J Surg. May 1988;155(5):663-6. [Medline].

Keywords

HCC, fibrolamellar carcinoma, fibrolamellar hepatoma, oncocytic hepatocellular carcinoma

Contributor Information and Disclosures

Author

W Ross Stevens, MD, Clinical Professor, Department of Radiology, Southern Illinois University School of Medicine; Head, Radiology Residency Director, Division of Gastrointestinal Radiology, St John's Hospital
W Ross Stevens, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Roentgen Ray Society, Illinois State Medical Society, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

John L Haddad, MD, Clinical Associate Professor, Department of Radiology, Weill Medical College of Cornell University; Director of Body MRI, Department of Radiology, Methodist Hospital in Houston
John L Haddad, MD is a member of the following medical societies: American College of Radiology, American Medical Association, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Udo P Schmiedl, MD, PhD, Clinical Professor, Department of Radiology, University of Washington; Consulting Staff, Swedish Medical Center, University of Washington Medical Center, Seattle Radiologists
Udo P Schmiedl, MD, PhD is a member of the following medical societies: American College of Radiology and Radiological Society of North America
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

John Karani, MBBS, FRCR, Consulting Staff, Department of Radiology, King's College Hospital, London
Disclosure: Nothing to disclose.

Further Reading