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Gut Online First, published on December 22, 2014 as 10.1136/gutjnl-2014-308726 Editor’s quiz: GI snapshot
Recurrent cholangitis in a 65-year-old man PART 1: CASE REPORT A 65-year-old man was referred for assessment of advanced liver disease. Seventeen years previously he had been diagnosed
Figure 2
Image of a section of the liver explant (H&E stain, ×1).
with ulcerative colitis, and 7 years later with primary sclerosing cholangitis (PSC). After several episodes with variceal bleeding caused by secondary biliary cirrhosis, a transjugular intrahepatic portosystemic shunt (TIPS) was created. Because of recurrent bacterial cholangitis, endoscopic retrograde cholangiography was performed, showing irregular narrowing of the intrahepatic bile ducts with saccular dilatations, findings considered compatible with PSC (figure 1). Also, a round filling defect in the common bile duct (CBD) was noted. After biliary sphincterotomy, a 6 mm polyp was removed. Histopathology review revealed a papillary adenoma with low-grade dysplasia. Cholangioscopy showed no evidence of residual polypous tissue or cancer. However, visualisation was difficult due to abundant mucus. Given the finding of a biliary adenoma against a background of PSC, the patient was considered to be at high risk for developing cholangiocarcinoma and was evaluated for liver transplantation. The operation took place in 2011 in the University Medical Centre, Ghent, Belgium. The pathology of the liver explant can be seen in figure 2.
Figure 1 Cholangiography showing irregular intrahepatic bile ducts, saccular dilatations and a 6 mm filling defect in the common bile duct. The previously positioned transjugular intrahepatic portosystemic shunt (TIPS) can be noted as well.
Question What is the diagnosis and the best therapeutic strategy for this condition?
Joosse ME, et al. Gut Month 2014 Vol 0 No 0
1
Copyright Article author (or their employer) 2014. Produced by BMJ Publishing Group Ltd (& BSG) under licence.
Downloaded from http://gut.bmj.com/ on February 17, 2015 - Published by group.bmj.com
Editor’s quiz: GI snapshot PART 2: ANSWER A more detailed image of the pathology of the liver explant can be seen in figure 3. Histological examination showed an intestinal-type intraductal papillary neoplasm of the intrahepatic biliary tree (figure 4) associated with a cholangiocarcinoma 1 cm in diameter in the distal CBD. No clear histological evidence for PSC was found. A final diagnosis of biliary papillomatosis was made. Biliary papillomatosis is a rare disease with approximately 150 reported cases. This disorder is characterised by multifocal papillary proliferations of the bile duct epithelium, resulting in multiple adenomas with high malignant potential.1 Patients often present with symptoms due to biliary obstruction caused by mucus.
Prolonged obstruction and cholestasis may result in secondary biliary cirrhosis and ultimately liver failure.2 Despite advances in diagnostic technology distinguishing, this disease from other inflammatory and neoplastic conditions remains challenging.3 Like in our case, patients are often misdiagnosed as having PSC. Abundant mucus during endoscopic retrograde cholangiography is strongly indicative for the diagnosis. Resection is a potential curative treatment for local disease but is controversial because of the high risk of recurrence.4 To improve poor prognosis, orthotropic liver transplantation should be considered as early as possible in the course of this rare disease. In this patient, liver transplantation was followed by a Whipple procedure with curative intent. The patient is alive 38 months after transplantation, although a lung metastasis has been removed by surgery 21 months after transplantation. M E Joosse,1 M E I Schipper,2 L Libbrecht,3 H R van Buuren,1 R A de Man1 1
Department of Gastroenterology and Hepatology, Erasmus MC, Rotterdam, The Netherlands 2 Department of Pathology, Erasmus Medical Center, Rotterdam, The Netherlands 3 Department of Pathology, Ghent University Hospital, Ghent, Belgium Correspondence to M E Joosse, Department of Gastroenterology and Hepatology, Erasmus Medical Center, P.O. Box 2040 Room 1567, Rotterdam 3000 CA, The Netherlands; [email protected] Contributors All authors have agreed with the submission of the manuscript in its present form. Competing interests None.
Figure 3 More detailed version of figure 2: Image of a section of the liver explant showing regeneration nodules (asterisk), concentric sclerosis around a bile duct (arrow) and intestinal-type intraductal papillary neoplasia (arrowhead) of a bile duct (H&E stain, ×1).
Provenance and peer review Not commissioned; externally peer reviewed. Data sharing statement Not relevant for this manuscript. To cite Joosse ME, Schipper MEI, Libbrecht L, et al. Gut Published Online First: [ please include Day Month Year] doi:10.1136/gutjnl-2014-308726 Received 27 October 2014 Revised 17 November 2014 Accepted 24 November 2014 Gut 2014;0:1–2. doi:10.1136/gutjnl-2014-308726
REFERENCES 1 2
3
4
Lee SS, Kim MH, Lee SK, et al. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer 2004;100:783–93. Imvrios G, Papanikolaou V, Lalountas M, et al. Papillomatosis of intra- and extrahepatic biliary tree: successful treatment with liver transplantation. Liver Transpl 2007;13:1045–8. Vassiliou I, Kairi-Vassilatou E, Marinis A, et al. Malignant potential of intrahepatic biliary papillomatosis: a case report and review of the literature. World J Surg Oncol 2006;4:71. Yeung YP, AhChong K, Chung CK, et al. Biliary papillomatosis: report of seven cases and review of English literature. J Hepatobiliary Pancreat Surg 2003;10:390–5.
Figure 4 Image of a section of the liver explant showing intestinal-type intraductal papillary neoplasm of an intrahepatic bile duct (H&E stain, ×20).
2
Joosse ME, et al. Gut Month 2014 Vol 0 No 0
Downloaded from http://gut.bmj.com/ on February 17, 2015 - Published by group.bmj.com
Recurrent cholangitis in a 65-year-old man M E Joosse, M E I Schipper, L Libbrecht, H R van Buuren and R A de Man Gut published online December 22, 2014
Updated information and services can be found at: http://gut.bmj.com/content/early/2014/12/22/gutjnl-2014-308726
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References
This article cites 4 articles, 0 of which you can access for free at: http://gut.bmj.com/content/early/2014/12/22/gutjnl-2014-308726#BIBL
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Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
Gut Online First, published on December 22, 2014 as 10.1136/gutjnl-2014-308726 Editor’s quiz: GI snapshot
Recurrent cholangitis in a 65-year-old man PART 1: CASE REPORT A 65-year-old man was referred for assessment of advanced liver disease. Seventeen years previously he had been diagnosed
Figure 2
Image of a section of the liver explant (H&E stain, ×1).
with ulcerative colitis, and 7 years later with primary sclerosing cholangitis (PSC). After several episodes with variceal bleeding caused by secondary biliary cirrhosis, a transjugular intrahepatic portosystemic shunt (TIPS) was created. Because of recurrent bacterial cholangitis, endoscopic retrograde cholangiography was performed, showing irregular narrowing of the intrahepatic bile ducts with saccular dilatations, findings considered compatible with PSC (figure 1). Also, a round filling defect in the common bile duct (CBD) was noted. After biliary sphincterotomy, a 6 mm polyp was removed. Histopathology review revealed a papillary adenoma with low-grade dysplasia. Cholangioscopy showed no evidence of residual polypous tissue or cancer. However, visualisation was difficult due to abundant mucus. Given the finding of a biliary adenoma against a background of PSC, the patient was considered to be at high risk for developing cholangiocarcinoma and was evaluated for liver transplantation. The operation took place in 2011 in the University Medical Centre, Ghent, Belgium. The pathology of the liver explant can be seen in figure 2.
Figure 1 Cholangiography showing irregular intrahepatic bile ducts, saccular dilatations and a 6 mm filling defect in the common bile duct. The previously positioned transjugular intrahepatic portosystemic shunt (TIPS) can be noted as well.
Question What is the diagnosis and the best therapeutic strategy for this condition?
Joosse ME, et al. Gut Month 2014 Vol 0 No 0
1
Copyright Article author (or their employer) 2014. Produced by BMJ Publishing Group Ltd (& BSG) under licence.
Downloaded from http://gut.bmj.com/ on February 17, 2015 - Published by group.bmj.com
Editor’s quiz: GI snapshot PART 2: ANSWER A more detailed image of the pathology of the liver explant can be seen in figure 3. Histological examination showed an intestinal-type intraductal papillary neoplasm of the intrahepatic biliary tree (figure 4) associated with a cholangiocarcinoma 1 cm in diameter in the distal CBD. No clear histological evidence for PSC was found. A final diagnosis of biliary papillomatosis was made. Biliary papillomatosis is a rare disease with approximately 150 reported cases. This disorder is characterised by multifocal papillary proliferations of the bile duct epithelium, resulting in multiple adenomas with high malignant potential.1 Patients often present with symptoms due to biliary obstruction caused by mucus.
Prolonged obstruction and cholestasis may result in secondary biliary cirrhosis and ultimately liver failure.2 Despite advances in diagnostic technology distinguishing, this disease from other inflammatory and neoplastic conditions remains challenging.3 Like in our case, patients are often misdiagnosed as having PSC. Abundant mucus during endoscopic retrograde cholangiography is strongly indicative for the diagnosis. Resection is a potential curative treatment for local disease but is controversial because of the high risk of recurrence.4 To improve poor prognosis, orthotropic liver transplantation should be considered as early as possible in the course of this rare disease. In this patient, liver transplantation was followed by a Whipple procedure with curative intent. The patient is alive 38 months after transplantation, although a lung metastasis has been removed by surgery 21 months after transplantation. M E Joosse,1 M E I Schipper,2 L Libbrecht,3 H R van Buuren,1 R A de Man1 1
Department of Gastroenterology and Hepatology, Erasmus MC, Rotterdam, The Netherlands 2 Department of Pathology, Erasmus Medical Center, Rotterdam, The Netherlands 3 Department of Pathology, Ghent University Hospital, Ghent, Belgium Correspondence to M E Joosse, Department of Gastroenterology and Hepatology, Erasmus Medical Center, P.O. Box 2040 Room 1567, Rotterdam 3000 CA, The Netherlands; [email protected] Contributors All authors have agreed with the submission of the manuscript in its present form. Competing interests None.
Figure 3 More detailed version of figure 2: Image of a section of the liver explant showing regeneration nodules (asterisk), concentric sclerosis around a bile duct (arrow) and intestinal-type intraductal papillary neoplasia (arrowhead) of a bile duct (H&E stain, ×1).
Provenance and peer review Not commissioned; externally peer reviewed. Data sharing statement Not relevant for this manuscript. To cite Joosse ME, Schipper MEI, Libbrecht L, et al. Gut Published Online First: [ please include Day Month Year] doi:10.1136/gutjnl-2014-308726 Received 27 October 2014 Revised 17 November 2014 Accepted 24 November 2014 Gut 2014;0:1–2. doi:10.1136/gutjnl-2014-308726
REFERENCES 1 2
3
4
Lee SS, Kim MH, Lee SK, et al. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer 2004;100:783–93. Imvrios G, Papanikolaou V, Lalountas M, et al. Papillomatosis of intra- and extrahepatic biliary tree: successful treatment with liver transplantation. Liver Transpl 2007;13:1045–8. Vassiliou I, Kairi-Vassilatou E, Marinis A, et al. Malignant potential of intrahepatic biliary papillomatosis: a case report and review of the literature. World J Surg Oncol 2006;4:71. Yeung YP, AhChong K, Chung CK, et al. Biliary papillomatosis: report of seven cases and review of English literature. J Hepatobiliary Pancreat Surg 2003;10:390–5.
Figure 4 Image of a section of the liver explant showing intestinal-type intraductal papillary neoplasm of an intrahepatic bile duct (H&E stain, ×20).
2
Joosse ME, et al. Gut Month 2014 Vol 0 No 0
Downloaded from http://gut.bmj.com/ on February 17, 2015 - Published by group.bmj.com
Recurrent cholangitis in a 65-year-old man M E Joosse, M E I Schipper, L Libbrecht, H R van Buuren and R A de Man Gut published online December 22, 2014
Updated information and services can be found at: http://gut.bmj.com/content/early/2014/12/22/gutjnl-2014-308726
These include:
References
This article cites 4 articles, 0 of which you can access for free at: http://gut.bmj.com/content/early/2014/12/22/gutjnl-2014-308726#BIBL
Email alerting service
Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
Topic Collections
Articles on similar topics can be found in the following collections GUT Snapshot (301)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
