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

Abstract

Previous research has confirmed that patients with choledochal cyst have an elevated risk of cholangiocarcinoma and gallbladder carcinoma. Current data suggest a risk of malignancy of 6 to 30% in adults with choledochal cyst. Malignancy has also occasionally been identified in children and adolescents. Multiple factors, including the age of the patient, cyst type, histological findings, and localization, have an impact on the prognosis. Information on long-term outcomes after cyst excision is limited. However, recent data suggest a lifelong elevated risk of up to 4% of cancer development following operation. This paper presents a review of the literature on cancer in patients with choledochal cyst before and after excision. A postoperative follow-up concept that consists of annual controls of CA19-9 and abdominal ultrasound is introduced.

Laparoscopic Choledochal Cyst Resection with Simplified Common Bile Duct Reconstruction in an Adult Population: A Case Series

“…26 The global incidence reported in the word medical literature for cholangiocarcinoma is 0.95 per 100,000, being more frequent in patients with CC. 21,22 The risk of malignancy reported for these patients is 0.7% to 28%, this risk increases over time. 2,5,6,8,23 Sastry et al, in one of their studies with a population of 7,880 patients (1,914 under 18 years of age and 3,866 adults), from which 4.59% had histopathological studies revealing malignancy, 70.4% of these patients had cholangiocarcinoma, 23.5% gallbladder carcinoma, and 6.1% other malignancies.…”

“…The incidence of cholangiocarcinoma in type I CC is 68%; and in type IV CC it is of 21%. 21 The risk of malignancy is high, and it is associated with a global survival of 6 to 21 years. Incidence of malignancy referred by Amid et al, in type IV CC was of 9.2%, type I 7.6%, type II 4.3%, type III 4%, and type V 2.5%.…”

Treatment of complex complications after choledochal cyst resection by multiple minimal invasive therapies: A case report

“…On the other hand, remnant choledochal cyst has been shown to increase the risk of malignancy. The mechanism of cancer development remains unclear, which may relate to inflammation, cell regeneration and DNA breaks caused by K-ras and p53 mutations [ 9 ]. Malignant cyst occurred mostly in type I and type IVa patients, followed by type V [ 9 , 10 ].…”

“…The mechanism of cancer development remains unclear, which may relate to inflammation, cell regeneration and DNA breaks caused by K-ras and p53 mutations [ 9 ]. Malignant cyst occurred mostly in type I and type IVa patients, followed by type V [ 9 , 10 ]. Both complications can affect patients’ quality of life as well as survival [ 11 ].…”

Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists

“…CCM can be classified anatomically according to the Todani classification [1] or its derivatives [2], based on morphological appearance and dilatation of the extrahepatic and/or intrahepatic bile ducts. CCM can also be considered a premalignant condition and one systematic review suggested that about 10% of all patients may develop a carcinoma of the biliary tract at some point in life, and usually at a younger age than the normal age of onset of bile duct cancer [3,4]. The long-accepted treatment therefore consists of excision of the entire extrahepatic biliary tree, followed by reconstruction with a Roux-en-Y hepaticojejunostomy.…”