S1926 A Young Female With Acute Cholangitis Due to Type IVa Choledochal Cyst With an Undifferentiated Spindle and Giant Cell Carcinoma Hiding Inside

Abstract

Introduction: This patient is being presented to demonstrate the rarity and severity of extrahepatic biliary cystic masses as the primary etiology of acute cholangitis in a young patient, after failing endoscopic stent placement. Case Description/Methods: A 29-year-old female with no medical history presented with 2 days of sharp, epigastric pain radiating to the back associated with fevers and non-bilious vomiting. Patient’s exam noted scleral icterus and abdominal exam showed tenderness to palpation in the right upper quadrant. Labs showed a WBC of 26,300, AST of 114, ALT of 289, ALP of 343 IU/L, and total bilirubin of 9.76 mg/dL. Computed tomography (CT) of the abdomen showed dilated proximal extrahepatic CBD with a large complex cystic mass in the porta hepatitis measuring 10 cm x 9 cm x 8 cm and this was contiguous with gallbladder and proximal common bile duct with mass effect on duodenum and pancreatic head concerning for Type I choledochal cyst. An ERCP showed a significantly dilated CBD with cystic dilation and balloon sweep with 9-10-12 mm balloon catheter was performed with passage of a small stone and pus. Two plastic stents 10 Fr x 7 cm and 10 Fr x 9 cm were placed with appropriate bile drainage. Fevers continued and total bilirubin increased to 14.2 mg/dL and ALP reached 535 IU/L, therefore patient was taken for laparoscopy which showed large choledochal cyst intimately involved with the head of the pancreas and duodenum, necessitating a Whipple procedure for distal margin. Proximal margin entered the left and the right hepatic ducts, consistent with a type IVa choledochal cyst. Anatomic pathology of the 7.0 cm x 6.0 cm x 3.0 cm mass in the cyst showed an undifferentiated spindle and giant cell carcinoma, confined to the bile duct histologically, with lymphovascular and perineural invasion present. There was no gallbladder involvement and 25 lymph nodes were negative for malignancy. The patient improved and was discharged to home after extended recovery (Figure). Discussion: Undifferentiated carcinoma of the extrahepatic biliary system is extremely rare and has been previously found on middle-age to elderly patients. Diagnosis of undifferentiated carcinoma of the extrahepatic biliary system is difficult because the exact diagnosis cannot be determined prior to surgical resection. Patients with undifferentiated carcinoma of the extrahepatic bile duct may have a relatively better prognosis due to the early appearance of jaundice, compared to those with gallbladder involvement.Figure 1.: MRCP of Type IV choledochal cyst