MR and MRCP in the Evaluation of Primary Sclerosing Cholangitis

Cholangiocarcinoma is the second most common primary malignant hepatobiliary neoplasm, accounting for approximately 15% of liver cancers. Diagnosis of cholangiocarcinoma is challenging and the prognosis is uniformly poor, with recurrence rates of 60%-90% after surgical resection. A wide spectrum of neoplastic and nonneoplastic conditions of the biliary tract may masquerade as cholangiocarcinoma, adding to the complexity of management in patients suspected to have cholangiocarcinoma. Mimics of cholangiocarcinoma constitute a heterogeneous group of entities that includes primary sclerosing cholangitis, recurrent pyogenic cholangitis, acquired immunodeficiency syndrome cholangiopathy, autoimmune pancreatitis, inflammatory pseudotumor, Mirizzi syndrome, xanthogranulomatous cholangitis, sarcoidosis, chemotherapy-induced sclerosis, hepatocellular carcinoma, metastases, melanoma, lymphoma, leukemia, and carcinoid tumors. These entities demonstrate characteristic histomorphology and variable clinicobiologic behaviors. The imaging findings of these disparate entities are protean and may be indistinguishable from those of cholangiocarcinoma. In most cases, a definitive diagnosis can be established only with histopathologic examination of a biopsy specimen. © RSNA, 2008 • After reading this article and taking the test, the reader will be able to: List the wide ■ spectrum of biliary tumors and pseudotumors that mimic cholangiocarcinoma. Describe the ■ cross-sectional imaging findings of neoplastic and nonneoplastic conditions of the biliary tract. Discuss how the ■ imaging features of radiologic mimics of cholangiocarcinoma correlate with the pathologic findings. Abbreviations: AIDS = acquired immunodeficiency syndrome, AIP = autoimmune pancreatitis, CCA = cholangiocarcinoma, ERCP = endoscopic retrograde cholangiopancreatography, H-E = hematoxylin-eosin, IPT = inflammatory pseudotumor, PSC = primary sclerosing cholangitis, RPC = recurrent pyogenic cholangitis, 3D = three-dimensional, XGC = xanthogranulomatous cholecystitis RadioGraphics 2008; 28:1115-1129 • Published online 10.1148/rg.284075148 • Content Codes:

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“…Arrowheads = associated biliary dilatation. the intra-and extrahepatic bile ducts (11,12) (Figs 6, 7).…”

Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

“…The classic imaging findings of PSC include multifocal strictures, segmental ectasias, ductal wall thickening, and irregular beading of the intra-and extrahepatic bile ducts (11,12) (Figs 6, 7).…”

Section: Page 1118mentioning

confidence: 99%

Mimics of Cholangiocarcinoma: Spectrum of Disease

et al. 2008

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“…The early stage is characterized by multiple annular or short segmental strictures (1-2 mm) with slightly dilated ducts among them which can create "beaded" appearance ( Fig. 4) [49,50]. Strictures are usually seen at the bile duct bifurcation.…”

Section: Diagnosis and Imaging Featuresmentioning

confidence: 99%

Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: an Update on MR Imaging Findings with Recent Developments

Kovač

Weber

2016

JGLD

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Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are the most common immune-mediated chronic cholestatic liver diseases leading to cirrhosis and liver failure. Although magnetic resonance imaging (MRI) is not a necessary procedure for the diagnosis of PBC, MRI is recommended for monitoring disease progression and early detection of complications. Even though liver cirrhosis subtypes have similar MR imaging features, there are some findings which could indicate PBC, such as the periportal halo sign. Additionally, MRI using diffusion-weighted imaging with apparent diffusion coefficient measurements provides non-invasive assessment of the stage of liver fibrosis. The role of cholangiography is crucial for the diagnosis of PSC. Since endoscopic retrograde cholangiography is an invasive procedure with occasional post-procedural complications, the latest guidelines suggest magnetic resonance cholangiography as a reference procedure for evaluation of patients suspected with PSC. Characteristic magnetic resonance cholangiography findings include multiple segmental strictures with slightly dilated ducts among them, usually on both intrahepatic and extrahepatic bile ducts. Furthermore, magnetic resonance cholangiography is useful in the follow-up of these patients, allowing for timely diagnosis of complications such as cholangiocellular carcinoma. With the exception of ursodeoxycholic acid, which slows the progression of PBC, the only curative treatment for both PSC and PBC is still liver transplantation. However, recurrent disease occurs in some patients indicating the need for development of new more effective therapies.

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“…Although ERCP allows intervention and biliary sampling and remains more sensitive for minor bile duct changes, 127 MRCP has established itself as the best initial safe and non‐invasive cholangiographic technique for diagnosis, with comparable accuracy to ERCP. It is cost‐effective 128 and it allows the demonstration of the biliary tree proximal to any obstruction, as well as when combined with contrast enhanced magnetic resonance imaging, giving additional information on extra‐ductal abnormalities 129 …”

Section: Primary Sclerosing Cholangitis: Background and Etiologymentioning

confidence: 99%

“…It is cost-effective 128 and it allows the demonstration of the biliary tree proximal to any obstruction, as well as when combined with contrast enhanced magnetic resonance imaging, giving additional information on extra-ductal abnormalities. 129 The etiology of PSC is unknown and it responds poorly, if at all, to immunosuppressive medications. 123 Overlap with autoimmune hepatitis in children is common, and a steroid responsive "autoimmune cholangiopathy" in association with AIH was apparent in half the cases of one study of children with primary AIH.…”

Section: Primary Sclerosing Cholangitis: Background and Etiologymentioning

confidence: 99%

New insights into autoimmune liver diseases

Kumagi

Alswat

Hirschfield

et al. 2008

Hepatology Research

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Autoinflammatory liver disease represents an important aspect of global hepatological practice. The three principal disease divisions recognized are autoimmune hepatitis, primary sclerosing cholangitis and primary biliary cirrhosis. Largely, but not exclusively, these diseases are considered to be autoimmune in origin. Increased recognition of outlier and overlap syndromes, changes in presentation and natural history, as well as the increased awareness of IgG4-associated sclerosing cholangitis, all highlight the limitations of the classic terminology. New insights continue to improve the care given to patients, and have arisen from carefully conducted clinical studies, therapeutic trials, as well as genetic and laboratory investigations. The challenges remain to treat patients before liver injury becomes permanent and to prevent the development of organ failure.

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MR and MRCP in the Evaluation of Primary Sclerosing Cholangitis

Cited by 33 publications

References 25 publications

Mimics of Cholangiocarcinoma: Spectrum of Disease

Mimics of Cholangiocarcinoma: Spectrum of Disease

Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: an Update on MR Imaging Findings with Recent Developments

New insights into autoimmune liver diseases

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