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Choledochal cyst is a premalignant condition of biliary tract.The development of malignancy in association withcholedochal cyst has been reported in 2.5-26% cases.[1] Sucha malignant change may occur anywhere in the biliary tract orin the pancreas. However, biliary papillomatosis rarelydevelops in a choledochal cyst and only two such cases havebeen reported so far.[2] We herein detail a case of biliarypapillomatosis with papillary adenocarcinoma in a patient withcholedochal cyst.

Case Report

A 50-year-male presented with fluctuating jaundice andcholestatic symptoms of 4 months duration. On examinationhe was jaundiced and had a 4 cm x 4 cm right-sided parietallump in the periumbilical region. The liver function tests wereconsistent with cholestatic jaundice. Abdominal ultrasoundrevealed dilated bile duct with intraluminal hyperechoic mass.Magnetic resonance cholangiography demonstrated Todanitype IVa choledochal cyst with an irregular intraluminal fillingdefect in the common bile duct, there was no evidence ofabnormal pacreato-biliary duct junction (Figure 1). Contrastenhanced computed tomography of the abdomendemonstrated dilated intrahepatic biliary radicles with localisedenhancing intraluminal mass in the supraduodenal commonbile duct and enhancing mass in relation to the right rectusmuscle. Fine needle aspiration cytology from the parietal walllump was inconclusive. In view of the localised nature of themass in the common bile duct, doubtful nature of parietal wallnodule and good performance status of patient, surgicalexploration was planned. Laparotomy revealed a dilated bileduct with a localised intraluminal soft tissue mass in thesupraduodenal part of the bile duct. There was no evidence ofregional lymphadenopathy. The parietal wall mass waslocalised to the right rectus muscle sparing the rectus sheathand peritoneum. The patient underwent complete excision ofthe extra-hepatic bile duct with Roux-en-Y-hepaticojejunostomy, excision of the right rectus muscle andnodule, and biopsy from both lobes of liver. The bile duct wasfilled with gelatinous mucoid material. The cut section of thebile duct revealed three soft, fleshy, cauliflower-shapedpedunculated masses with wide stalks. The frozen sectionsfrom the proximal and distal margins were free of tumour. Oneof the masses measured 6 cm x 6 cm and the remaining twowere 2 cm x2 cm in size. The parietal mass was greyish, hardand gritty with a variegated appearance and measured 4 cm x5 cm. Microscopic examination of the bile duct growthsrevealed papillomas with connective tissue stroma and frond-like branches lined with columnar epithelium. The largestgrowth had several foci of moderate to severe nuclear atypiaand an area of moderately differentiated papillaryadenocarcinoma with invasion of cyst wall and periductularneural plexus (Figure 2). Sections from the remaining cyst revealed absence of mucosa in most parts and evidence ofmixed inflammatory infiltrate. The resection margins of thecyst and pericholedochal lymph nodes were free of tumour.Sections from the parietal wall nodule revealed papillaryadenocarcinoma and liver biopsy showed evidence ofextensive hepatic fibrosis. Postoperatively the patientdeveloped ascites and wound infection, which responded wellto conservative measures. The patient was discharged twoweeks later and was doing well up until four and a half yearsafter surgery when he developed diffuse bilateral hepaticmetastasis and died.





Discussion

Biliary papillomatosis is an uncommon clinicopathologicalentity characterised by multiple papillary adenomas of thebiliary epithelium. Such lesions may involve any part of thebiliary tract including the gall bladder. These lesions producelarge amount of jelly-like mucus, which may lead to intermittentbiliary obstruction and cholangitis.3 Although histologically categorised as benign, biliary papillomatosis should beconsidered a premalignant lesion as malignant transformationhas been observed in 35% of cases at the time of presentationor on follow-up.[4] Biliary papillomatosis may demonstrate notonly dysplastic changes but also progression to carcinoma-in-situ and frankly invasive adenocarcinoma.[5] Hence, it hasbeen suggested that the adenoma-carcinoma sequence  maybe seen in these lesions.[4] The median age at diagnosis inreported series of patients with biliary papillomatosis is 58years with slight male preponderance and the majority ofpatients present with intermittent obstructive jaundice.[4] maging studies may reveal dilatation of the biliary tree withmultiple ill-defined and fuzzy filling defects or intraluminal frond-like mass lesions.[6] Curettage and drainage procedures areassociated with recurrence in almost all the patients.[4] Radicalsurgery in the form of bile duct excision,pancreaticoduodenectomy and hepatectomy is the treatmentof choice7 and is associated with 14% local recurrence rate.[4] After complete surgical excision with pathologically clearmargins, local recurrence can still occur in the remnant biliarytract.[7] Distant metastasis has rarely been reported with biliarypapillomatosis.[7,8] n fact, 5-year survival was observed in apatient with biliary papillomatosis with abdominal wall andpulmonary metastasis after surgical excision.[7]

Biliary papillomatosis has been rarely reported inassociation with choledochal cysts and only 2 such caseshave been reported so far.[2,9] Mutation of K-ras and the p53gene has been suggested as the underlying mechanism inthe pathogenesis of these lesions.[2,9] Although not present inthis case, anomalous pancreato-biliary duct junction may alsoplay a role in themalignant change in these lesions.[2] To thebest of our knowledge, biliary papillomatosis with papillaryadenocarcinoma arising in choledochal cyst has not beenreported so far. The treatment of choice for choledochal cyst iscomplete excision of the extrahepatic bile duct withhepaticojejunostomy.

References

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3. Styne P, Warren GH, Kumpe DA, Halgrimson C, Kern F. Obstructivecholangitis secondary to mucus secreted by a solitary papillarybile duct tumor. Gastroenterology. 1986;90:748–53.
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