Table of Contents    
Case Report
 
Intrahepatic cholangiocarcinoma with congenital hepatic fibrosis
Adeel S. Zubair1, Ali Metwaly2, Justin M. Burns3, John A. Stauffer4
1Medical Student, Mayo Medical School, Rochester, Minnesota, USA.
2Observing Physician, Mayo Clinic Jacksonville, Jacksonville, Florida, USA.
3Surgeon, Department of Transplantation, Mayo Clinic Jacksonville, Jacksonville, Florida, USA.
4Surgeon, Department of Surgery, Mayo Clinic Jacksonville, Jacksonville, Florida, USA.

Article ID: 100043IJHPDAZ2015
doi:10.5348/ijhpd-2015-43-CR-18

Address correspondence to:
John Stauffer
MD, 4500 San Pablo Road
Jacksonville
FL 32224
Phone: 904-953-2214
Fax: 904-953-7368

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How to cite this article:
Zubair AS, Metwaly A, Burns JM, Stauffer JA. Intrahepatic cholangiocarcinoma with congenital hepatic fibrosis. Int J Hepatobiliary Pancreat Dis 2015;5:103–107.


Abstract
Introduction: Little is known regarding the association between congenital hepatic fibrosis (CHF) and cholangiocarcinoma and publications reporting these associations are rare. In literature, only four cases reporting the associations exist; those four cases were all diagnosed at autopsy. Herein, we describe a case of CHF and intrahepatic cholangiocarcinoma that was successfully treated with surgical resection.
Case Report: A 46-year-old male with past medical history significant for CHF for 12 years, complicated by portal hypertension and esophageal varices, was found to have lesions on MRI suspicious for intrahepatic cholangiocarcinoma. The patient had relatively preserved liver function, and the decision was made to proceed with surgical resection. Pathology showed poorly differentiated cholangiocarcinoma with background features of CHF.
Conclusion: All patients with CHF patients should be monitored for the development of cholangiocarcinoma. If the patient has compensated disease, treatment via partial hepatectomy is preferred. With vigilant screening and appropriate treatment, it is possible to decrease morbidity and mortality from the development of intrahepatic cholangiocarcinoma in the setting of CHF.

Keywords: Cholangiocarcinoma, Cirrhosis, Congenital hepatic fibrosis


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Author Contributions
Adeel S. Zubair – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Ali Metwaly – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Justin M. Burns – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
John A. Stauffer – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2015 Adeel S. Zubair et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.