Abdominal Imaging
ª Springer Science+Business Media, Inc. 2005 Published online: 10 August 2005
Abdom Imaging (2005) 30:744–747 DOI: 10.1007/s00261-005-0334-4
Follicular cholangitis mimicking hilar cholangiocarcinoma J. Y. Lee, J. H. Lim, H. K. Lim Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong, Kangnam-ku, Seoul 135-710, Korea
Abstract A 61-year-old man with intermittent periumbilical pain and high liver enzyme levels underwent spiral computed tomography of the abdomen with intravenous contrast medium enhancement. Abdominal computed tomogram showed wall thickening of both intrahepatic ducts and proximal common hepatic duct with enhancement. Direct cholangiogram showed severe smooth strictures from the common hepatic duct to both intrahepatic ducts. The findings were interpreted as hilar cholangiocarcinoma. The pathologic findings after surgical resection were consistent with follicular cholangitis. Key words: Cholangitis—Benign biliary stricture—Hilar cholangiocarcinoma
Differentiating benign stricture of the bile ducts from malignant disease by using nonsurgical methods is sometimes difficult. Segmental stricture of the bile ducts found in patients with no history of biliary tract disease should be considered as cholangiocarcinoma. Several investigators have reported cases in which patients underwent surgical exploration for a preoperative diagnosis of cholangiocarcinoma that turned out to be benign disease [1, 2]. In this report, we describe a case of follicular cholangitis presenting as a segmental stricture of the hepatic ducts and common bile duct that was considered to be hilar cholangiocarcinoma but which was proved on pathologic examination to be a benign stricture with formation of lymph follicles with germinal centers.
Case report A 61-year-old man presented with intermittent periumbilical pain and red urine for 20 days. He visited a local
Correspondence to: J. H. Lim; email:
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hospital, and sonography and computed tomography (CT) revealed segmental stricture of the common hepatic duct; common bile duct cancer was suspected. He was diagnosed with gallbladder stones. He had been treated for intermittent right upper quadrant pain for 3 years under the diagnosis of gallbladder stones. Recently, the symptom became more aggravated and his sclerae turned yellow. He previously underwent left upper lobectomy for pulmonary tuberculosis. He had no history of abdominal surgery. On physical examination, he was afebrile and there was no tenderness in the upper abdomen. The sclerae were icteric. Laboratory data on admission showed a normal white blood cell count (7.66 · 103/lL) but a high erythrocyte sedimentation rate (64 mm/h) and high levels of total bilirubin (5.8 mg/dL), direct bilirubin (3.4 mg/ dL), aspartate aminotransferase (54 U/L), alanine aminotransferase (124 U/L), alkaline phosphatase (178 U/ L), amylase (161 U/L), and lipase (552 U/L). Abdominal ultrasonography disclosed dilatation of the intrahepatic ducts and narrowing of the right and left hepatic ducts and proximal common hepatic duct. A small gallbladder stone was seen, and the walls of the proximal common hepatic duct and gallbladder were slightly thick. Abdominal CT with iodinated contrast material administration showed wall thickening of both hepatic ducts and the proximal common hepatic duct with enhancement (Fig. 1A, B), resulting in diffuse segmental stenosis. The lesion extended to the cystic duct and to parts of the gallbladder neck. A small stone was seen in the intrapancreatic portion of the distal common bile duct. There were several small lymph nodes around the common bile duct. CT angiography showed no invasion into the hepatic artery and portal vein. The patient underwent biliary drainage from the bile duct of Couinaud segment III of the liver. Direct cholangiography through the biliary drainage tube revealed severe smooth stricture from the common hepatic duct to both intrahepatic ducts (Fig. 1C). Thus, under a diagnosis of hilar cholangiocarcinoma, the patient underwent surgery.
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Fig. 1. A 62-year-old man with intermittent right upper abdominal pain for 2 months and recent development of jaundice. Jaundice is relieved after insertion of a drainage catheter into a bile duct of the left hepatic lobe. A CT scan during arterial phase shows obliteration of the right and left hepatic ducts due to concentric wall thickening (arrows). B CT scan displays concentric wall thickening of the common hepatic duct with contrast enhancement (arrow). C Tube chol-
angiogram demonstrates severe narrowing of the right and left hepatic ducts (arrows) and common hepatic duct. A catheter was inserted into the common hepatic duct (arrowhead) through one of the dilated left hepatic ducts. D Microphotograph of a resected wall of the common hepatic duct shows uniform (straight arrow) or granular (arrowhead) wall thickening. Many lymphoid follicles are embedded within the thickened wall, which are the hallmark of follicular cholangitis.
In the operative field, there was no mass, but an adhesive change between the common bile duct, cystic duct, and gallbladder was observed. Frozen biopsy showed chronic inflammatory cell infiltration. Resection of the extrahepatic bile duct and part of the right intrahepatic duct, cholecystectomy, and hepaticojejunostomy
were performed. Anastomosis between the right and left intrahepatic ducts was performed and followed by insertion of a short segment of silastic stent into the right intrahepatic duct. The mucosa of the extrahepatic bile duct of the resected specimen showed coarse granulation and its wall
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was concentrically thickened to 0.5 to 0.7cm. The right intrahepatic duct showed the same finding of granular mucosa and thickened wall. The left intrahepatic duct showed a clear mucosal surface and mild wall thickening measuring 0.2 to 0.4 cm. The histologic feature revealed a benign granular luminal growth with remarkable formation of lymphoid follicles. The submucosa of the bile duct showed smooth muscle hyperplasia, diffuse neural hypertrophy, and diffuse lymphoid follicle formation. The pathologic findings were consistent with follicular cholangitis.
Discussion Benign bile duct stricture may result from trauma and chronic inflammation such as sclerosing cholangitis. In contrast, isolated biliary strictures occurring at the hepatic duct confluence or within the extrahepatic ducts in patients without a history of surgery or stone disease are usually due to cholangiocarcinoma [1, 3]. Accurate preoperative diagnosis can permit appropriate therapeutic planning with potential early resection for malignant disease. The preoperative assessment is of limited usefulness for excluding malignancy because of its low sensitivity (30% to 60%) [4, 5]. Although a positive diagnosis is a reliable indicator of malignancy, a negative result does not exclude malignancy [2, 4]. Noninvasive imaging techniques such as sonography and CT play an important role in the initial evaluation of biliary strictures. These studies can be used to identify mass lesions or stones and intrahepatic bile duct dilatation. A segment of stricture longer than 14 mm is highly suggestive of malignancy [6]. Direct cholangiography (percutaneous transhepatic cholangiography or endoscopic retrograde cholangiopancreatography) is useful in delineating the degree and extent of stenosis. Benign biliary strictures are characteristically short and ring-like, whereas malignant strictures are usually long and irregular. However, it is often impossible to differentiate malignancy from benignancy. In some patients, vessel encasement and occlusion on hepatic arteriography may be an indication of malignancy. In other patients, however, benign strictures of the intrahepatic bile duct are occasionally associated with arterial involvement. Endoscopic retrograde wire-guided brushing is useful in discriminating malignant from benign strictures of the extrahepatic biliary tract. In most instances, however, a neoplastic stricture may be impossible to exclude, even with multiple biopsies [2, 4, 5]. In our case, percutaneous cholangiogram through a biliary drainage tube revealed a segmental stricture of both hepatic ducts extending down to the proximal common hepatic duct. Even though there was no malignancy in the cytology of bile, the lesion was strongly considered to be a hilar cholangiocarcinoma. Pathologic examinations revealed that the stricture was a
benign inflammation consisting of lymph follicles with active germinal centers and focal hyalinization. The results were similar to those of previous reports [3, 7]: According to these reports, the mucosa of the common bile duct of follicular cholangitis shows a moderate stenosis by granular nodules that have a ‘‘cobblestone-like’’ appearance. These granular nodules were represented as granular filling defects on cholangiogram. Our case showed diffuse, smooth narrowing of the bile ducts on cholangiogram and irregular thickening of the bile ducts with contrast enhancement on CT, mimicking periductalinfiltrating cholangiocarcinoma. The etiology of follicular cholangitis is unknown. Histologic and immunohistochemical examinations are not pathognomonic and suggest only that there is a longstanding inflammatory condition involving the bile ducts [3, 7]. Various stimuli can cause polyclonal hyperplasia of lymph follicles around the bile ducts. Similar histologic findings have been observed in the bronchiole [4, 8], which is called follicular bronchiolitis. The histologic findings of follicular bronchiolitis are characterized by peribronchiolar infiltrates composed of a mixture of lymphocytes, plasma cells, and germinal centers, which in turn cause compression of the lumen in some terminal bronchioles. The disease is sometimes associated with collagen vascular disease, immunodeficiency, or hypersensitivity reaction. Our patient had no history of autoimmune disease and there were no antinuclear antibodies. In summary, we have described a case of follicular cholangitis mimicking hilar cholangiocarcinoma on radiologic findings. Irregular thickening of the bile ducts and granular appearance on cholangiogram suggested follicular cholangitis. In the differential diagnosis of follicular cholangitis, primary sclerosing cholangitis, periductal infiltrating or sclerosing cholangiocarcinoma, stricture associated with hepatobiliary lithiasis, and lymphoma of bile duct should be included. In primary sclerosing cholangitis, the classic features on cholangiography are the beading appearance of the bile ducts with various strictures of different length and decreased arborization [9]. Differentiating sclerosing cholangiocarcinoma from follicular cholangitis is more difficult. The granular appearance on cholangiogram and irregular wall thickening of the bile ducts on CT may be differential findings from periductal-infiltrating cholangiocarcinoma. These findings represent hypertrophied submucosal lymphoid follicles on pathology of follicular cholangitis. Bile duct stricture in patients with recurrent pyogenic cholangitis and stones usually involves a short segment with a tight stenosis. Stones are usually depicted by using sonography, CT, and cholangiography. Lymphoma of the bile duct is an extremely rare disease, and cholangiography may demonstrate stricture of the common bile duct and sonography may reveal a hypoechoic mass filling the lumen of the bile duct [10].
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