Intrahepatic Cholangiocarcinoma Arising 33 Years After Excision of a Choledochal Cyst: Report of a Case

Abstract

We report a case of intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst. A 61-year-old woman was admitted to our hospital complaining of fever. Thirty-three years ago she had undergone extrahepatic choledochal cystectomy and choledochojejunostomy for a choledochal cyst. Computed tomography showed a tumor in the anterior segment of the liver, extending to the posterior and medial segments and the right portal vein. Intrahepatic biliary stones were seen in the bile ducts. We performed extended right lobectomy. Microscopically, the tumor was cholangiocarcinoma. Most of the tumor area was composed of invasive adenocarcinoma but a carcinoma-in-situ component was also observed in some regions including the hilar bile duct, where an intrahepatic biliary stone was seen. This suggests that the cancer development could be related to intrahepatic cholestasis. Patients with choledochal cyst may have to be carefully followed up for more than 30 years even after diversion surgery.

Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia

“…It is recommended that the extrahepatic bile duct be resected near the level of the pancreatobiliary junction with a wide hepaticoenterostomy at the hilum of the liver to avoid these complications . Development of biliary cancer following choledochal cyst resection, cholangitis, and intrahepatic stones have been noted predominantly in types I and IV‐A cysts in several studies …”

“…22 Development of biliary cancer following choledochal cyst resection, cholangitis, and intrahepatic stones have been noted predominantly in types I and IV-A cysts in several studies. 12,15,22,27 Proposed timeline: From birth to transition/transfer. Children with CDCs are typically diagnosed within the first decade of life.…”

Choledochal Cyst and Malignancy: A Plea for Lifelong Follow-Up

“…[57][58][59] In several series, all patients with malignancy following cyst excision were diagnosed with adenocarcinoma (►Table 1). 28,29,38,40,42,58 Recently, Ng et al reported on the rare case of metachronous cholangiocarcinoma 13 years postresection and Tajiri et al described a mucinous cystadenoma of the pancreas 17 years following gallbladder resection due to choledochal cyst. 57,60 Otani et al described a rare case of a bile duct schwannoma developing in remnant choledochal cyst.…”

Choledochal cysts in adults: A case report and review of literature

“…[7] Technically, cyst excision is the main step to diminish the risk for cancer development, and must be performed when it is possible. [3] Unfortunately, a cholangiocarcinoma may arise even after the cyst excision, as described by Nishiyama et al [8] The surgical strategy depends on the type of the cyst. For the Type I cyst, complete excision of the cyst is usually feasible and Roux-en-Y hepaticojejunostomy is the preferable reconstruction technique.…”