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ABDOMINAL RADIOLOGY Table of Contents   
Year : 2008  |  Volume : 18  |  Issue : 3  |  Page : 236-238
Case report: Cholangiocarcinoma in a choledochal cyst


Department of Radiodiagnosis, Command Hospital Air Force, Bangalore, India

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   Abstract 

Cholangiocarcinoma is a dreaded complication of unresected choledochal cysts, with an incidence approaching 20-30% in early adulthood. The risk of cholangiocarcinoma remains high where an internal drainage procedure has been performed and the cyst has been partially resected or left unresected. We report a case of cholangiocarcinoma occurring in an unresected choledochal cyst following a drainage procedure in infancy and highlight the role of PET/CT in its diagnosis.

Keywords: Choledochal cyst; cholangiocarcinoma; PET/CT; biliary anomalies

How to cite this article:
Chaturvedi A, Singh J P, Rastogi V. Case report: Cholangiocarcinoma in a choledochal cyst. Indian J Radiol Imaging 2008;18:236-8

How to cite this URL:
Chaturvedi A, Singh J P, Rastogi V. Case report: Cholangiocarcinoma in a choledochal cyst. Indian J Radiol Imaging [serial online] 2008 [cited 2019 Oct 21];18:236-8. Available from: http://www.ijri.org/text.asp?2008/18/3/236/41836
Cholangiocarcinoma is a dreaded complication of unresected choledochal cysts, with an incidence approaching 20-30% in early adulthood. [1] The risk of cholangiocarcinoma remains high where an internal drainage procedure has been performed and the cyst has been partially resected or left unresected. [1],[2] We report a case of cholangiocarcinoma occurring in an unresected choledochal cyst following a drainage procedure in infancy and highlight the role of PET/CT in its diagnosis.


   Case History Top


A 20-year-old unmarried girl reported to the surgical outpatient department with complaints of unrelenting pain in the upper abdomen, vomiting, and loss of weight for 2 months. There was a history of some surgery done for jaundice in early infancy but the details were not available. Clinical examination revealed a jaundiced, cachexic patient, with a rounded mass in the right upper quadrant that was separate from the liver. She was anemic and biochemical tests for liver function revealed a cholestatic type of jaundice.

USG revealed a large cystic lesion containing air and debris in the subhepatic region and a lobulated soft tissue mass along the anterior wall of this cyst inferiorly [Figure 1A]. Another separate, 2.5-cm, rounded solid mass was seen in the region of the pancreatic head [Figure 1B]. The common bile duct (CBD) was not separately visualized and there was pneumobilia, with dilatation of the proximal intrahepatic biliary radicles. CT scan revealed a large cystic mass completely replacing the CBD, with reflux of orally ingested iodinated contrast into the cyst as well as the intrahepatic biliary radicles, suggesting a biliary-enteric communication. The intracystic soft tissue mass and the pancreatic head mass showed significant enhancement [Figure 2]. MRCP showed the cystic duct entering the cyst, confirming it to be a dilated CBD [Figure 3]. The intracystic soft tissue showed up as large filling defects along the anteroinferior cyst wall.

On the basis of these findings, the diagnosis of a type 1 choledochal cyst, with evidence of a prior cysto-duodenal bypass surgery and the presence of an intracystic cholangiocarcinoma, was made. The patient was unwilling for image-guided fine needle aspiration cytology. A PET/CT performed to further evaluate the intracystic mass showed markedly increased tracer uptake within this soft tissue as well as in the pancreatic head mass (SUV 4.5). Additional peritoneal deposits were also detected in the left lower quadrant and pelvis [Figure 4]. This reaffirmed the suspected diagnosis of cholangiocarcinoma along with intraabdominal spread. The patient was advised chemotherapy; unfortunately, he left the hospital against medical advice and was lost to follow-up.


   Discussion Top


Choledochal cysts are more prevalent in Asia than in Western countries and approximately 33-50% of reported cases are from Japan. [3] They occur more often in women, with a male to female ratio of 1:3 to 4. [1] Todani's classification of choledochal cysts is at present universally accepted and is as follows: [4]

Type I - Dilatation of hepatic and common bile ducts (40-85%)

Type II - CBD diverticulum (2-4%)

Type III - Intraduodenal CBD dilatation (1.4-5.6%)

Type IV - a Intrahepatic and extrahepatic bile duct dilatation (18-20%)

Type IV - b Multiple extrahepatic cysts (rare)

Type V - Intrahepatic bile duct dilatation (rare)

The classic clinical triad of abdominal pain, jaundice, and abdominal mass is found only in a minority of patients. The clinical presentation largely depends on age - abdominal pain being the most frequent presenting symptom in adults and jaundice in infants. [1] In most patients, USG suffices for the detection and diagnosis of a choledochal cyst. A typical choledochal cyst appears as a water-density mass at the porta or adjacent to the head of the pancreas, with varying degrees of intrahepatic biliary dilatation. [5] A cross-sectional modality such as CT and MRI is usually indicated preoperatively to delineate its relationship with the surrounding structures and also when malignancy is suspected. [1],[5] MRCP is increasingly being accepted as a safe, noninvasive substitute for ERCP. [6] It allows direct imaging of the cyst in multiple planes and can demonstrate its communication with the biliary tract. It can also demonstrate any anomaly of the pancreaticobiliary duct junction, which is often considered a possible etiological factor in choledochal cyst formation. [1],[6]

Complications of choledochal cysts are the result of stasis and include cholangitis, stone formation, recurrent pancreatitis, cirrhosis, and portal hypertension. Cholangiocarcinoma is a dreaded complication and is attributed to chronic mucosal irritation; it has an incidence of 10-30%. This risk is even higher after drainage procedures. [1],[2] Nodular wall thickening and/or an enhancing mass in a choledochal cyst are highly suspicious of malignant change. [5],[7] In these high-risk patients, FDG PET/CT may detect early tumors and reveal unsuspected distant metastases and thus impact management. [8]

Because of the high and persistent risk of cholangiocarcinoma, primary excision of extrahepatic choledochal cysts with biliary-enteric anastomosis is the treatment of choice in all cases, even in the absence of symptoms. [1],[2] Cholangiocarcinoma developing in a choledochal cyst usually has an adverse outcome because of late diagnosis and a low possibility of resectability.

 
   References Top

1.de Vries JS, de Vries S, Aronson DC, Bosman DK, Rauws EA, Bosma A, et al . Choledochal cysts: Age of presentation, symptoms, and late complications related to Todani's classification. J Pediatr Surg 2002;37:1568-73.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Jordan PH Jr, Goss JA Jr, Rosenberg WR, Woods KL. Some considerations for management of choledochal cysts. Am J Surg 2004;187:790-5.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Miyano T, Yamataka A. Choledochal cysts. Curr Opin ≠Pediatr 1997;9:283-8.  Back to cited text no. 3  [PUBMED]  
4.Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts, classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977;134:263-9.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Haaga JR, Herbenen TE. The gall bladder and biliary tree. In : Haaga JR, Lanzieri CF, editors. CT and MR Imaging of the Whole Body. 4 th ed. St Louis, Missouri: Mosby; 2003. p. 1362-4.  Back to cited text no. 5    
6.Fitoz S, Erden A, Boruban S. Magnetic resonance cholangiopancreatography of biliary system abnormalities in children. Clin Imaging 2007;31:93-101.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Yoshida H, Itai Y, Minami M, Kokubo T, Ohtomo K, Kuroda A. Biliary malignancies occurring in choledochal cysts. Radiology 1989;173:389-392  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Slattery JM, Sahani DV. What is the current state-of-the-art imaging for detection and staging of cholangiocarcinoma? Oncologist 2006;11:913-22.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Arti Chaturvedi
Department of Radiodiagnosis, Command Hospital Air Force, Airport Road, Bangalore - 560 007
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.41836

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    Figures

  [Figure 1A], [Figure 1B], [Figure 2], [Figure 3], [Figure 4]

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