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The nodularity associated with cirrhosis is

typically relatively fine and diffuse rather

than coarse and lobulated [2]. However, non-

cirrhotic causes of fine, diffuse nodularity are

occasionally encountered and include pseudo-

cirrhosis of treated breast cancer metastases

to the liver, fulminant hepatic failure, miliary

metastases, and sarcoidosis [3–6].

Pseudocirrhosis of Treated Breast Cancer

 Metastases to the Liver 

A variety of hepatic contour changes can oc-

cur in breast cancer patients with metastases to

the liver after chemotherapy including fine, dif-

fuse nodularity that resembles cirrhosis (Fig.

1), which is commonly referred to as “pseudo-

cirrhosis” [3]. Multifocal retraction of the liv-

er capsule and enlargement of the caudate

lobe also can be seen in this setting. The his-

topathologic or pathophysiologic basis of these

changes is not well understood, and the prefix

“pseudo-” may be a misnomer because these

patients can develop features of portal hyper-

tension such as portosystemic venous collater-

als and bland ascites (Figs. 1 and 2). Why thisphenomenon seems almost specific for breast

cancer (Fig. 3) remains unclear, with only spo-

radic reports of these changes in other primary

malignancies with liver metastases [7].

Fulminant Hepatic Failure

Fulminant hepatic failure constitutes acute

severe impairment of hepatic function in the ab-

sence of preexisting liver disease and receives

higher priority for hepatic transplantation than

Radiologic Mimics of Cirrhosis

Priyanka Jha1

Liina Poder

Zhen J. Wang

Antonio C. Westphalen

Benjamin M. Yeh

Fergus V. Coakley

Jha P, Poder L, Wang ZJ, Westphalen AC, Yeh BM,Coakley FV

1All authors: Department of Radiology, University of

California, San Francisco, 505 Parnassus Ave., Box 062 8,

M-372, San Francisco, CA 94143-0628 . Addresscorrespondence to F. V. Coakley (Fergus.Coakley@

radiology.ucsf.edu).

CME

This article is available for CME credit.

See www.arrs.org for more information.

Gastrointest inal Imaging • Pictorial Essay

AJR  2010; 194:993–9 99

0361–803X/10/1944–993

© American Roentgen Ray Society

CT of patients with established cir-

rhosis shows diffuse surface nod-

ularity of the liver and also may

show decreased liver volume with

relative hypertrophy of the left and caudate

lobes or signs of portal hypertension. When

seen at imaging, diffuse hepatic surface nodu-

larity or signs of portal hypertension (i.e.,

splenomegaly, ascites, or portosystemic va-

rices) are usually due to cirrhosis, but other

conditions can also cause these findings.

Knowledge of radiologic mimics of cirrhosis

is increasingly important because some have

been described only recently and because an

erroneous diagnosis of cirrhosis in some set-

tings could adversely impact contemporary

treatment options. For example, underlying

cirrhosis lowers the transplantation status of

patients with acute hepatic failure. This article

aims to provide a practical and current review

of the conditions other than cirrhosis that can

result in diffuse hepatic surface nodularity or

signs of portal hypertension.

Noncirrhotic Causes of DiffuseHepatic Surface Nodularity

General Comments

Diffuse irregularity of the hepatic contour

may be fine or coarse (Appendix 1). Causes

of coarse lobulation include chronic Budd-

Chiari syndrome, chronic portal vein throm-

bosis, and pseudomyxoma peritonei [1]. The

hepatic contour changes due to these condi-

tions usually are easily distinguishable from

cirrhosis because of characteristic features.

Keywords: cirrhosis, liver, liver disease, pseudocirrhosis

DOI:10.2214/AJR.09.3409

Received July 30, 200 9; accepted after revision

September 15, 2009 .

Z. J. Wang was supported by an NIBIB training grant

(1 T32 EB001631).

OBJECTIVE. The objective of this article is to provide a practical review of the condi-

tions other than cirrhosis that can result in diffuse surface nodularity of the liver or portal

hypertension.

CONCLUSION. Conditions that can mimic cirrhosis on imaging include pseudocirrho-

sis of treated breast cancer metastases to the liver, fulminant hepatic failure, miliary metasta-

ses, sarcoidosis, schistosomiasis, congenital hepatic fibrosis, idiopathic portal hypertension,

early primary biliary cirrhosis, chronic Budd-Chiari syndrome, chronic portal vein thrombo-sis, and nodular regenerative hyperplasia.

Jha et al.Radiologic Mimics of Cirrhosis

Gastrointestinal ImagingPictorial Essay

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hepatic failure developing in the setting of cir-

rhosis. The distinction of acute from acute-on-

chronic hepatic failure may be clinically diffi-

cult and hepatic surface nodularity might be

considered indicative of underlying cirrhosis,

adversely impacting transplantation status. Re-

cently, fulminant hepatic failure alone was

shown to result in diffuse surface nodularity be-cause of a combination of alternating foci of

confluent regenerative nodules and necrosis

rather than cirrhosis [8] (Figs. 4 and 5).

 Miliary Metastases

Diffuse surface nodularity due to miliary

metastases is rare (Figs. 6 and 7) and is un-

likely to mimic cirrhosis. In a large ultra-

sound study [4] of hepatic metastases (n  =

225), surface irregularity was observed in 16

patients (7%); in only two patients, one with

squamous cell carcinoma of the tonsil and one

with ovarian carcinoma, was surface nodular-

ity the only sign of metastatic disease [4].

Sarcoidosis

Sarcoidosis of the liver is common patho-

logically but is rarely visible at imaging be-

cause the noncaseating granulomas are usu-

ally microscopic. Occasionally, hepatic

sarcoidosis can be visible as diffuse granular

heterogeneity with or without fine nodularity

of the hepatic surface [5] (Figs. 8 and 9).

Noncirrhotic Causes of Portal

Hypertension

General CommentsNoncirrhotic causes of portal hyperten-

sion (Appendix 2) include chronic Budd-

Chiari syndrome, chronic portal vein throm-

bosis, sarcoidosis, schistosomiasis, nodular

regenerative hyperplasia, congenital hepatic

fibrosis, idiopathic portal hypertension, and

early primary biliary cirrhosis [9].

Nodular Regenerative Hyperplasia

Nodular regenerative hyperplasia is a rare

but increasingly recognized condition char-

acterized by widespread transformation of

normal liver parenchyma into hyperplastic

regenerative nodules that vary in size frommicroscopic to large and masslike [10]; the

absence of fibrosis distinguishes nodular re-

generative hyperplasia from cirrhosis. From

a radiologic perspective, there appear to be

two forms of the condition: a diffuse form in

which the nodules are small and a widespread

and a focal form in which the nodules are few

in number, are scattered throughout the liver,

and measure up to a few centimeters.

The pathogenesis of diffuse nodular regen-

erative hyperplasia is unknown, but there are

well-recognized associations (Appendix 3)

with systemic cardiovascular, myeloprolifer-

ative, and autoimmune diseases, particularly

systemic lupus erythematosus; the administra-

tion of certain drugs including chemotherapy;

solid organ and bone marrow transplantation;and HIV infection [10]. Focal nodular regener-

ative hyperplasia has been primarily reported

in long-standing Budd-Chiari syndrome, al-

though similar masses have also been report-

ed in autoimmune hepatitis [11, 12]. Diffuse

nodular regenerative hyperplasia frequent-

ly results in noncirrhotic portal hypertension

[10], and affected patients may present with

variceal bleeding or hypersplenism.

Imaging findings in patients with nodular

hyperplasia can be subtle across all techniques,

although parenchymal findings may be more

obvious on ultrasound than on CT or MRI [13].

At ultrasound, widespread nodularity sugges-

tive of cirrhosis or multiple masses may be

seen (Fig. 10). At CT, the nodules may be hy-

podense with little enhancement. At MRI, dif-

fuse nodular regenerative hyperplasia is usual-

ly of similar signal intensity to the liver on T1,

T2, and gadolinium-enhanced sequences. Sur-

face nodularity and features of portal hyper-

tension may be seen across all techniques (Fig.

11). In focal nodular regenerative hyperplasia,

CT or MRI typically shows multiple hypervas-

cular masses in a patient with long-standing

Budd-Chiari syndrome or autoimmune hepa-

titis. These masses may suggest metastases orhepatocellular carcinoma, although stability on

follow-up studies or biopsy should help in mak-

ing the correct diagnosis.

Early Primary Biliary Cirrhosis

Investigators recently found that portal

hypertension can develop early in prima-

ry biliary cirrhosis—even before the onset

of cirrhosis [14]—possibly resulting from

compression of the portal venous branch-

es by granulomatous inflammation causing

presinusoidal fibrosis and portal hyperten-

sion [14]. This condition has been described

as the portal hypertensive variant of primarybiliary cirrhosis and appears to carry a high-

er risk of hepatocellular carcinoma than the

other variants of primary biliary cirrhosis.

Congenital Hepatic Fibrosis

Hepatic involvement in this multisys-

tem autosomal recessive disorder consists

of widespread periportal fibrosis leading to

portal hypertension [15]. Portal vein throm-

bosis is common, and segmental biliary dila-

tation may also be observed (Fig. 12).

Idiopathic Portal Hypertension

Idiopathic portal hypertension is character-

ized by long-standing presinusoidal portal hy-

pertension of unknown cause in adults and may

reflect damage to the intrahepatic small portalveins or portal tracts by an immunologic dis-

turbance, thromboembolism, or an infection.

Imaging findings (Fig. 13) include subcapsular

parenchymal atrophy, portal and parenchymal

fibrosis, and portal venous thrombosis. Inter-

estingly, cirrhosis does not develop even in the

advanced stages of the disease [16].

Conclusion

Diffuse surface nodularity of the liver or

signs of portal hypertension usually reflect un-

derlying cirrhosis, but noncirrhotic causes of

these imaging findings include pseudocirrhosis

of treated breast cancer metastases to the liver,

fulminant hepatic failure, miliary metastases,

sarcoidosis, schistosomiasis, congenital hepat-

ic fibrosis, idiopathic portal hypertension, early

primary biliary cirrhosis, chronic Budd-Chiari

syndrome, chronic portal vein thrombosis, and

nodular regenerative hyperplasia.

Pseudocirrhosis of treated breast cancer me-

tastases can be easily identified by reviewing

the patient’s medical history and prior imaging

examinations. Pseudocirrhosis of fulminant

hepatic failure should be considered in previ-

ously healthy patients with acute liver decom-

pensation. Miliary metastases should be con-sidered when fine hepatic surface nodularity is

seen in a patient with a known primary malig-

nancy and no signs of portal hypertension. A

raised serum angiotensin-converting enzyme

level or mediastinal adenopathy suggests sar-

coidosis. Congenital hepatic fibrosis should be

considered in children with findings of portal

hypertension, particularly if there are cysts in

the kidneys. A smooth liver of normal size with

features of portal hypertension should raise sus-

picion for idiopathic portal hypertension, but

biopsy may be required because this diagno-

sis is one of exclusion. Diffuse nodular regen-

erative hyperplasia should be considered whenmultiple small masses are seen in the liver of a

patient with known risk factors, particularly if

the clinical picture does not favor malignancy

and if the lesions are more prominent at ultra-

sound than on other techniques. Focal nodular

regenerative hyperplasia should be considered

when multiple hypervascular hepatic masses

are seen in a patient with long-standing Budd-

Chiari syndrome or autoimmune hepatitis.

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Although these distinguishing features may

be helpful, histologic correlation will be required

to diagnose some cases. Careful radiologic anal-

ysis and clinical correlation may be required to

prevent an erroneous diagnosis that could po-

tentially adversely impact management.

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APPENDIX 1: Noncirrhotic Causes of Diffuse Hepatic Contour Irregularity

Coarse lobulation

Chronic Budd-Chiari syndrome

Chronic portal vein thrombosis

Pseudomyxoma peritonei

Fine nodularity

Pseudocirrhosis of treated breast cancer metastases to the liver

Fulminant hepatic failure

Miliary metastases

Sarcoidosis

APPENDIX 2: Noncirrhotic Causes of Portal Hypertension

1. Chronic Budd-Chiari syndrome

2. Chronic portal vein thrombosis

3. Sarcoidosis

4. Schistosomiasis

5. Nodular regenerative hyperplasia

6. Congenital hepatic fibrosis

7. Idiopathic portal hypertension

8. Early primary biliary cirrhosis

APPENDIX 3: Associations of Diffuse and Focal Forms of Nodular Regenerative Hyperplasia

Diffuse formCardiovascular diseases

Myeloproliferative diseases

Autoimmune diseases, particularly systemic lupus erythematosus

Certain drugs including chemotherapy

Solid organ and bone marrow transplantation

HIV infection

Focal form

Chronic Budd-Chiari syndrome

Autoimmune hepatitis

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A

Fig. 1—62-year-old woman with breast cancer treated with chemotherapy.A, Axial contrast-enhanced CT image obtained after patient had received chemotherapy treatment shows diffuse surface nodularity in liver and recanalized umbilicalvein (arrow ); these findings are suggestive of cirrhosis.B, Axial contrast-enhanced CT image obtained 6 months before A, which was before patient started chemotherapy, shows multiple hepatic metastases. Liver isotherwise normal. Setting of breast cancer metastases treated with chemotherapy indicates rapid development of diffuse changes seen in A likely representspseudocirrhosis of treated breast cancer.

B

A

Fig. 2—59-year-old woman with multiple hypodense biopsy-proven hepatic metastases from invasive ductal carcinoma of breast.A, Axial contrast-enhanced CT image obtained before patient started chemotherapy.B, Axial contrast-enhanced CT image obtained 6 months after A—that is, after patient had started chemotherapy—shows diffuse hepatic nodularity, bland ascites(asterisk ), esophageal varices (arrow ), and partial regression of hepatic metastases. Findings are of pseudocirrhosis of treated breast cancer metastases; however,without prior studies and clinical history, these findings could suggest diagnosis of cirrhosis.

B

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A

Fig. 3—68-year-old woman who presented for imaging after receiving two cycles of chemotherapy for hepatic metastases thought to be from primary pancreaticcarcinoma.A, Axial contrast-enhanced CT image shows liver surface is coarsely lobulated with several irregular hypodense parenchymal lesions. Appearance was consideredsuggestive of pseudocirrhosis, casting doubt on diagnosis of pancreatic cancer.B, Axial contrast-enhanced image obtained at more superior level than A shows small hypervascular lesion (arrow ) in right breast. Further workup including resection ofbreast mass confirmed diagnosis of metastatic breast cancer.

B

A

Fig. 4—61-year-old man with fulminant hepaticfailure and history of chronic hepatitis B infection.A, Contrast-enhanced CT image shows nodularity(arrow ) of liver surface outlined by ascites; therefindings are suggestive of cirrhosis. Histopathologicexamination of explanted liver 5 days later showedconfluent regenerative nodules surrounded by largeareas of necrosis, but no cirrhosis. Hepatic surfacenodularity is not a reliable sign of underlying cirrhosisin fulminant hepatic failure and should not be used todiagnose cirrhosis in this setting.B, Photomicrograph of explanted hepatic surfacefrom transplant surgery performed 5 days afterA shows that irregularity of liver surface reflectscombination of confluent regenerative nodules (R)and alternating bands of necrosis (N). (H and E, ×200)

B

Fig. 5—67-year-old woman with fulminant hepatic failure that developed 6 weeks after commencement ofmethyldopa therapy for hypertension. Sagittal ultrasound image of right hepatic lobe shows nodularity (arrow )of liver surface outlined by ascites; there findings are suggestive of cirrhosis. Histopathologic examinationof explanted liver 3 days later showed confluent regenerative nodules surrounded by large areas of subacutenecrosis but no cirrhosis.

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Fig. 6—64-year-old woman with metastatic lobular breast cancer. Axial contrast-enhanced CT image shows fine nodularity of hepatic surface. Liver biopsyrevealed metastatic breast cancer without cirrhosis.

Fig. 7—58-year-old woman with bilateral lobular breast cancer. Axial contrast-enhanced CT image shows widespread diffuse parenchymal and surface hepaticnodularity. Biopsy revealed metastatic disease without cirrhosis.

Fig. 8—32-year-old man with sarcoidosis. Axial contrast-enhanced CT imageshows widespread diffuse parenchymal and surface hepatic nodularity (arrow ).Appearance of liver on CT alone could be interpreted as cirrhosis, but notemultiple hypodense nodules in spleen. Retroperitoneal adenopathy (not shown)

was also present. Nodal biopsy confirmed diagnosis of sarcoidosis.

Fig. 9—44-year-old man with sarcoidosis. Axial unenhanced CT image showssplenomegaly (asterisk ) and recanalized umbilical vein (arrow ) arising fromsomewhat shrunken and irregular liver. Liver biopsy revealed sarcoidosis withoutcirrhosis. Sarcoidosis is one cause of noncirrhotic portal hypertension.

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F O R Y O U R I N F O R M A T I O N

 This artic le is avai lable for CME c redit. See w ww.a rrs.org for more inform ati on.

Fig. 11—60-year-old man with portal hypertension leading to gastrointestinalbleeding, ascites, and thrombocytopenia due to biopsy-proven nodularregenerative hyperplasia in liver transplant; transplantation was performed 18years earlier for primary sclerosing cholangitis. Axial contrast-enhanced CTimage shows liver surface (arrow ) is irregular and shows relative hypertrophy ofleft hepatic lobe (asterisk ). These findings mimic those of cirrhosis.

Fig. 12—11-year-old girl with renal failure due to autosomal recessive polycystickidney disease. Coronal T2-weighted MR image shows kidneys (K) are replacedby innumerable relatively small cysts. Focal segmental biliary dilatation (arrow )in liver reflects coexistent congenital hepatic fibrosis, which can occur inassociation with autosomal recessive polycystic kidney disease and is cause ofnoncirrhotic portal hypertension. Note spleen (asterisk ) is enlarged.

A

Fig. 10—58-year-old man with history ofrenal transplantation for HIV nephropathywho presented with sepsis 1 day after righthemicolectomy for colonic volvulus.A, Sagittal ultrasound image of righthepatic lobe shows subtle echogenicnodularity of liver that could be consideredsuggestive of cirrhosis. Representativenodule (arrow ) is visible anteriorly.

B, Axial contrast-enhanced CT imageshows subtle parenchymal heterogeneityconsisting of small hypodense nodules.Representative nodule (arrow ) isvisible posteriorly. Subsequent liverbiopsy confirmed diagnosis of nodularregenerative hyperplasia.

B

Fig. 13—68-year-old man with idiopathic portal hypertension. Axial contrast-enhancedCT image shows splenomegaly (S), gastroesophageal varices (white arrow ), and ascites(black arrow ), but liver appears normal in size and contour. Liver biopsy confirmedabsence of cirrhosis; final diagnosis was idiopathic portal hypertension.