Cholangiocarcinoma in a 21-year-old male with pancreaticobiliary malunion and choledochal cyst: A case report

Abstract

Significance:
Choledochal cysts and pancreaticobiliary malunion are rare conditions. Both are highly correlated with biliary tract cancer. Its predisposition to malignant transformation is due to chronic pancreaticobiliary reflux and inflammation leading to dysplasia and carcinoma.

Clinical Presentation:
A 21-year-old male presented with three months’ duration of epigastric pain and no known risk factors. Initial ultrasound imaging noted an ill-defined lesion at the left hepatic lobe and concomitant dilated intrahepatic and biliary ducts.

Management:
Considering a liver abscess, ultrasound-guided drainage of abscess was done along with complete antibiotic regimen. Repeat imaging showed a Type IVa choledochal cyst and persistent left hepatic lobe lesion. CA 19-9 was markedly elevated. ERCP confirmed presence of choledochal cyst and a concomitant pancreaticobiliary malunion with high insertion of pancreatic duct about 1.6 cm away from the ampulla. Biliary stenting was done. Highly suspecting malignant transformation associated with choledochal cysts, diagnostic laparoscopy was done revealing whitish peritoneal implants. Final
histopathological diagnosis was conclusive of metastatic cholangiocarcinoma. Thereafter he was then referred to oncology service. Unfortunately, after three months, the patient expired.

Recommendations:
In a young patient with recurring and worsening abdominal pain associated with presence of choledochal cyst and evidence of biliary tract anatomical malunion, a possible biliary tract malignancy should be highly suspected. Reporting this anomaly when discovered at imaging is important for close follow-up of patients at risk.