CHOLEDOCHAL CYST TO CHOLANGIOCARCINOMA

Abstract

Case Presentation: A 35 year old Caucasian female presented with abdominal pain for one month. The pain was intermittent, dull, and nonradiating, and associated with nausea and vomiting. The patient also reported dark amber urine but denied fevers or chills. Physical exam revealed mild right upper quadrant abdominal pain and hepatomegaly. Laboratory data revealed: Hemoglobin 12.1, INR0.9, Total bilirubin 2.7, Direct bilirubin 1.2, AST317, ALT 700, Alkaline phosphatase 563, and lipase 592. An abdominal CT showed dilated left and right intrahepatic ducts but no extrahepatic dilation. No focal masses were seen in the liver. ERCP showed a three centimeter duodenal stricture and a single, long, irregular common bile duct stricture from the distal common bile duct to the bifurcation. Brush cytology from the common bile duct was negative but duodenal biopsies were positive for poorly differentiated adenocarcinoma. Between the ages of five and six, the patient experienced intermittent jaundice. Extensive workup revealed a choledochal cyst. She underwent an ex- ploratory laparotomy and choledochoduodenostomy. A generous portion of the wall of the cyst was excised at the time of surgery. Surgical exploration revealed an umbilical mass, which histologically was consistent with metastatic adenocarcinoma. Palliative surgery was completed, and treatment with 5-fluorouracil, leucovorin, and localized radiation therapy was initiated. Due to the inability to tolerate therapy, the patient opted to defer any further treatment. Comfort measures were instituted, and the patient was discharged to hospice. Discussion: Choledochal cysts are biliary cystic dilatations, which may occur singly or in multiples throughout the bile ducts. The incidence of biliary cysts is estimated to be 1:100,000 to 150,000 with higher incidence rates in some Asian nations. The female to male ratio is approximately 3:1. Cysts may be congenital or acquired; however, the exact pathogenesis is unknown. Types I, II, and III biliary cysts include choledochal cysts. Types IV and V may include intrahepatic and/or extrahepatic cystic dilatations. Common presentations include chronic and intermittent abdominal pain, intermittent jaundice, and recurrent cholangitis. Direct cholangiography is the best test for diagnosis and evaluation. Because of the risk of malignant degeneration of the cyst, surgical excision is the current standard, with the goal of removing all of the cyst tissue when possible.