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 19 June 2018

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Marek Hartleb, Jan Baron and Piotr Paleń Early cancer in congenital choledochal cyst
Marek Hartleb, Jan Baron and Piotr Paleń, 14 April 2008


Congenital choledochal cysts are associated with significantly increased risk of development of bile duct cancer. Unfortunately, in many patients the cancer is diagnosed in non-resectable stage of its growth. Therefore, the standard approach to choledochal cysts is surgical resection before cancer develops. We present a 43-year-old female in whom well-differentiated flat adenocarcinoma was found in cystic wall on postsurgery histopathological examination.

Case report


A 43-year-old woman was admitted to the hospital because of several episodes of pain in the right hypochondrium associated with dark urine and subfebrile state. Her clinical history was uneventful apart from jaundice of unknown origin occurring 25 years earlier.


On admission the laboratory data showed marked non-icteric cholestasis (alkaline phosphatase 235 IU/l, GGTP 743 IU/) with moderate hypertransaminasemia (ALT 134 IU/l, AST 75 IU/l). Serum level of CA19-9 was 24,7 IU/ml (normal values below 24,0 IU/mL) and that of CEA was within normal range.

Magnetic resonance cholangiography (Figure 1) and spiral computed tomography (Figure 2) disclosed cystic dilations of common bile duct and intrahepatic portion of left hepatic duct. The choledochal cyst had clear margins and homogenous consistency.

The diagnosis was congenital cystic bile duct dilatations (Todani's type IV-A) and the patient was sent to surgery.

Figure 1 Magnetic resonance cholangiography showing disclosed cystic dilations of common bile duct and intrahepatic portion of left hepatic duct.

Figure 2 Spiral computed tomography showing cystic dilations of common bile duct and intrahepatic portion of left hepatic duct


The choledochal cyst was excised and Roux-en-Y hepatico-jejunostomy was performed. The postoperative period was not complicated. The histology showed normal biliary duct epithelium, but in the site of spotty mucosal discoloration a tiny well-differentiated tubular adenocarcinoma was found (Figure 3).

Figure 3 Normal biliary duct epithelium with a tiny well-differentiated tubular adenocarcinoma.


Choledochal cysts in adults are rare, being 3 to 4 times more common in females than males. Biliary cysts are usually symptomatic with cholestasis and right upper quadrant pain as the leading symptoms. Choledochal cysts are associated in adults with a 14% overall incidence of cholangiocarcinoma, mostly showing a poor prognosis [1]. Therefore, it is unusual to find in the wall of choledochal cyst a small cancer undetectable on imaging methods [2].

This article was first published on on 14 April 2008.


1. Dr Marek Hartleb MD, PhD
2. Dr Jan Baron MD, PhD
3. Dr Piotr Paleń MD

Departments of Gastroenterology1, Radiology2 and Pathomorphology3, Medical University of Silesia, Katowice, Poland

Contact information

Dr Marek Hartleb MD, PhD
Department of Gastroenterology
Medical University of Silesia


  1. Chapman RW. Risk factors for biliary tract carcinogenesis. Ann Oncol 1999;10 Suppl 4: 308-11.
  2. Kraus I, Rubinic M, Uravic, et al. Early cancer in congenital choledochal cyst. Coll Antropol 2003; 27: 677-83.

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