Primary congenital choledochal cyst with squamous cell carcinoma: a case report

Abstract

Cases of extrahepatic bile duct carcinoma are mostly adenocarcinomas and extrahepatic bile duct squamous cell carcinomas are rare. We report here a case of choledochal squamous cell carcinoma in a young woman who underwent surgery and chemotherapy. The woman presented with abdominal discomfort. A physical examination showed tenderness in the upper abdomen. Laboratory tests showed elevated direct bilirubin, total bilirubin, and C-reactive protein levels. Abdominal computed tomography and magnetic resonance imaging showed a cystic-solid mixed soft tissue mass in the common bile duct. Pain symptoms in the patient were not relieved and surgical treatment was performed. Postoperative pathological results showed a choledochal cyst complicated by squamous cell carcinoma. The patient was treated by biliary intestinal anastomosis followed by chemotherapy. However, the patient developed liver metastasis and recurrence at a 6-month follow-up. Primary congenital bile duct cysts with squamous cell carcinoma are extremely rare. Surgical resection is the main treatment option for choledochal squamous cell carcinoma. Postoperative chemoradiotherapy can be used, but the efficacy is poor and chemotherapy does not significantly prolong the patient’s survival.

Rare primary squamous cell carcinoma of the intrahepatic bile duct: A case report and review of literature

“…Third, the metaplastic squamous epithelium of the bile duct has the potential to transform into SCC, and the inflammation associated with choledochal cysts, cholelithiasis, sclerosing cholangitis, and Caroli disease could be an important cause of metaplasia[ 17 ]. Some experts have suggested that the “inflammation-cancer” transformation is an inescapable risk factor for the occurrence of cholangiocarcinoma, although SCC is rarer than adenocarcinoma[ 18 ]. Fourth, ectopic squamous epithelium may be another etiology.…”

“…Moreover, ICC adenocarcinoma has delayed enhancement in the venous phase, sometimes accompanied by dilation of the distal bile duct on CT or magnetic resonance imaging. However, biliary SCC may present as an avascular and delayed or heterogeneous enhancement mass similar to that of the ICC adenocarcinoma[ 18 , 20 ] (Table 1 ). Our patient’s CT manifestations revealed an avascular and heterogeneous enhancement mass.…”

Comparison of open and laparoscopic outcomes of adult Type-I congenital choledochal cysts

“…1 The condition is characterized by spindle-shaped and cystic dilatation of the common bile duct, and, in some patients, intrahepatic bile duct dilatation, which can lead to abnormal confluence of bile and pancreatic ducts, poor drainage of bile and pancreatic juices, repeated biliary tract infections, biliary stones, biliary pancreatitis, and even cancer. 2 Therefore, in adults, congenital choledochal cysts should be treated in a timely manner by surgically removing the cyst lesions and then reconstructing the biliary tract.…”