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BMC Surgery
Published in: BMC Surgery 1/2017

Open Access 01-12-2017 | Case report

Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report

Authors: Narongsak Rungsakulkij, Pattana Sornmayura, Penampai Tannaphai

Published in: BMC Surgery | Issue 1/2017

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Abstract

Background

The most common cause of perihilar obstruction is cholangiocarcinoma, especially in Thailand. Benign perihilar stricture represents less than 20% of cases. IgG4-related disease and IgG4-related sclerosing cholangitis, however, have been receiving increased recognition. Isolated IgG4-related sclerosing cholangitis is less common. The preoperative diagnosis of IgG4-related sclerosing cholangitis without pancreatic involvement is very difficult because the clinical presentation and preoperative evaluation are extremely difficult to distinguish from perihilar cholangiocarcinoma.

Case presentation

We report the case of a 56-year-old man who presented with obstructive jaundice with preoperative imaging showing proximal common bile duct obstruction. He underwent right lobe liver hepatectomy with extrahepatic bile duct resection and regional lymph node dissection due to high suspicion of malignancy. The pathological report showed severe acute and chronic inflammation of the bile duct with morphology and immunohistochemistry suggestive of IgG4-related sclerosing cholangitis.

Conclusions

IgG4-related sclerosing cholangitis with perihilar obstruction should be considered even in areas where cholangiocarcinoma is endemic.
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Metadata
Title
Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report
Authors
Narongsak Rungsakulkij
Pattana Sornmayura
Penampai Tannaphai
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Surgery / Issue 1/2017
Electronic ISSN: 1471-2482
DOI
https://doi.org/10.1186/s12893-017-0214-1

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