Intrahepatic cholangiocarcinoma arising 34 years after excision of a type IV-A congenital choledochal cyst: Report of a case

Abstract

We report a rare case of intrahepatic cholangiocarcinoma (IHCC) arising many years after excision of a type IV-A congenital choledochal cyst. A 44-year-old man was transferred to our hospital with acute cholangitis more than 34 years after several operations for congenital biliary dilatation. Imaging showed a huge tumor in the left medial section of the liver, extending to the porta hepatis. Although he had no jaundice, the intrahepatic bile ducts showed cylinder-like dilatation with narrowing of the hilar bile duct. At surgery, the tumor was found to arise from the dilated intrahepatic bile duct just above the narrow portion. He underwent a left hepatic trisectionectomy with a vascular procedure. Microscopically, the tumor was confirmed to be moderate-to-well-differentiated tubular adenocarcinoma. Thus, when the narrow segment is left untouched, careful long-term follow-up is important to detect new lesions at an early stage.

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

“…5 Second, the existence of stenosis at the anastomosis site or narrow segment in the intrahepatic bile duct may independently induce carcinogenesis even if there was no progression to a precancerous stage at the time of diversion surgery. 8,13 Last, cancer can develop de novo in the general population. Our patient underwent diversion surgery in our institute 33 years before the discovery of advanced cancer, but detailed medical records, including preoperative status of the biliary system (e.g., Todani's classification of the choledochal cyst), 14 were not preserved.…”

“…If a stenosis at the anastomosis site, a narrow segment in the intrahepatic bile duct, or an intrahepatic biliary stone is found, treatment for improving biliary stasis or strict follow-up using imaging devices and blood examination may be necessary, as suggested in past reports. 8,13 We may have to accept biannual examination of enhanced computed tomography and blood test in the patients with potential risk. If the patient has no abnormalities related to the presence of intrahepatic cholestasis, the necessity for long-term follow-up is unknown, but we believe that lifelong follow-up is still an option for confirming a patient's current condition after diversion surgery.…”

“…1,5 Cases of cancer development in the biliary system more than 10 years after diversion surgery have been reported. 1,5,[9][10][11][12][13] However, it is unknown whether such patients had carcinogenic potential for several decades after diversion surgery. Cancer could develop postoperatively due to cholestasis or it could develop de novo in patients after diversion surgery, as can occur in healthy individuals.…”

Role of thymidylate synthase and dihydropyrimidine dehydrogenase mRNA in intrahepatic cholangiocarcinoma

“…The incidence of IHCC has been reported in only about 3.63% of primary liver carcinoma cases [1]. Its incidence in Japan has increased in the past decade, and curative surgery is considered to be the only truly effective treatment [2][3][4][5][6]. However, patients with IHCC have a poorer prognosis than those with other malignancies, even if curative resection has been performed [7][8][9][10].…”

Intrahepatic cholangiocarcinoma with intrahepatic biliary lithiasis arising 47 years after the excision of a congenital biliary dilatation: Report of a case

“…According to the literature, 2.5%-28% of CBD cases are associated with malignant biliary tract tumors at the initial presentation (2). The cause of this carcinogenesis is presumed to be the reflux of pancreatic juice into the bile duct caused by an anomalous junction of the pancreatobiliary duct.…”