Abstract
Cystic dilatations of the cystic duct which are suggested as type VI biliary cysts are very rare and many of them go unrecognized or are confused with other cysts until the operation although they are obvious on imaging studies. They can present with fusiform or saccular dilatations and can be accompanied by common bile duct dilatations. It is important to identify these cysts as they share the same characteristics as the other biliary cyst types and can be complicated with malignancy. We herein present a very unusual case of a cholangiocarcinoma arising from a type VI biliary cyst in a 58-year-old female patient and review the literature. The patient presented with jaundice, weight loss, and abdominal pain. On imaging, the cystic duct and common bile duct were fusiformly dilated and had a wide communication. There was a mass filling the distal parts of both ducts. The patient was urgently operated on after perforation following ERCP. Histopathology was compatible with a type VI biliary cyst and an associated cholangiocarcinoma.
Highlights
Choledochal cysts are congenital cystic dilatations of any portion of the bile ducts which are not associated with a tumor, stone, or inflammation as the cause of the dilatation [1]
It was not until that all the findings of fusiform dilatation of the cystic and choledochal ducts were interpreted as a cystic duct cyst which was proposed as a type VI biliary cyst
Biliary cysts are classified into five categories as described by Todani et al Type I is cyst of the CBD; type II is a cystic diverticulum of the extrahepatic bile duct; type III is a cyst in the intraduodenal portion of the common bile duct; type IV refers to multiple cysts in the intra- and extrahepatic biliary tract; and type V is single or multiple cysts in the intrahepatic ducts alone which is known as Caroli disease [2]
Summary
Choledochal cysts are congenital cystic dilatations of any portion of the bile ducts which are not associated with a tumor, stone, or inflammation as the cause of the dilatation [1]. Congenital cystic duct cysts are extremely rare with a very limited number of cases in the literature. They are regarded as “type VI biliary cysts” and the term was first proposed by Serradel et al [3]. It is well known that there is an association of bile duct adenocarcinoma and biliary cysts [4]. Adenocarcinoma arising from a type VI biliary cyst is extremely rare with only one reported case in the literature [5]. We aimed to present the imaging findings of an adenocarcinoma arising from a type VI biliary cyst which is the second case in the literature to our knowledge and review the literature
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