Concomitant hilar cholangiocarcinoma with choledochal cyst and cholelithiasis in an asymptomatic patient: A case report

INTRODUCTION AND IMPORTANCE: Choledochal cyst is an important risk factor for cholangiocarcinoma. Concomitant hilar cholangiocarcinoma with choledochal cyst with cholelithiasis is a rare finding. CASE PRESENTATION: A 52-year-old male presented with non-specific complaints of generalized weakness for 3 months. Transabdominal ultrasound showed dilated common bile duct with hyperechoic mass at hilar region; further evaluation with magnetic resonance cholangiopancreatography and contrast-enhanced computer tomography of abdomen and pelvis revealed concomitant intraductal hilar cholangiocarcinoma (Bismuth Corlette type 1) with Choledochal cyst (type IVa) with cholelithiasis. After optimization patient underwent left hepatectomy with common bile duct excision with cholecystectomy with Roux-en-Y hepaticojejunostomy. Histopathological examination confirmed it to be well-differentiated adenocarcinoma, intestinal type at the hilar confluence. CLINICAL DISCUSSION: An asymptomatic male patient with the concomitant finding of perihilar cholangiocarcinoma (Bismuth Corlette type I) with choledochal cyst type IVa with cholelithiasis is a rare finding. The patient was managed with surgical excision of the common bile duct with Roux-en-Y hepaticojejunostomy and cholecystectomy. Diagnostic evaluation should be proper not to miss or overlook such a synchronous lesion. CONCLUSION: Incidental finding of concomitant perihilar cholangiocarcinoma with the choledochal cyst is rare. Proper evaluation of the patient with CECT abdomen and pelvis and MRCP is necessary for the diagnosis. Proper surgical resection with adequate lymph node removal is important for surgical clearance.