Potential Risk Factors and Prognostic Evaluation of Malignant Changes Following Congenital Choledochal Cyst: a Retrospec
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ORIGINAL ARTICLE
Potential Risk Factors and Prognostic Evaluation of Malignant Changes Following Congenital Choledochal Cyst: a Retrospective Analysis Huaqin Guan 1 & Renpin Chen 1 & Dan Li 1 & Rukhsaar Hoosen 2 & Saili Xie 3 & Chao Chen 1 & Sisi Jin 1 Received: 24 June 2019 / Accepted: 19 March 2020 # Association of Surgeons of India 2020
Abstract Congenital choledochal cyst (CCC) is a congenital anomaly of the biliary duct with a tendency for malignant evolution. This study aimed to investigate the potential risk factors involved in the malignant transformation of these cysts and to elucidate the survival status of patients who subsequently developed malignancy. One hundred and fifty patients were involved in the study. They were divided into two groups; people in group A had malignancy, while people in group B did not. Of the total 150 patients, 15 (10%) developed malignancy. On univariate analysis, significant differences were found between the two groups, showing an increasing risk with older age (p = 0.003), presence of jaundice (p = 0.000) and biliary calculi (p = 0.000), and elevated conjugated bilirubin (CB), alkaline phosphatase (ALP), and γ-glutamyl transpeptidase levels (p = 0.005, 0.020, 0.021). On logistic regression analysis, only older age (p = 0.036), presence of biliary calculi (p = 0.003), and elevated levels of CB and ALP were identified as significant factors (p = 0.000, 0.039). In group A, the median overall survival time was 28.8 ± 8.1 months (95% confidence interval 12.9–44.7). In summary, older age, presence of biliary calculi, and elevated levels of CB and ALP are important indicators of the potential malignant transformation of CCC. Actively treating patients with biliary obstruction and impaired liver function are effective management methods for patients diagnosed with CCC. Keywords Congenital choledochal cyst . Malignancy . Risk factors . Survival analysis
Introduction Congenital choledochal cyst (CCC) is a congenital anomaly of the biliary ducts characterized by cystic dilatation of the extraand/or intrahepatic biliary ducts [1, 2]. Most cases are diagnosed during early childhood, but between 20 and 30% of cases are first detected in adults [3]. A choledochal cysts carry up to an approximately 10% risk of malignant transformation to cholangiocarcinoma [4]. Malignancy in association with CCC was first reported by Irwin and Morison in 1944 [5]. They reported that the total * Sisi Jin [email protected] 1
Department of Gastroenterology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325000, China
2
Wenzhou Medical University, Wenzhou 325000, China
3
Department of Ultrasound, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325000, China
malignancy rate of CCC was 2.5–28%, which was 25–40 times higher than that of the normal population. Voyles et al. [6] in their study of 1433 patients with CCC found that the incidence of malignancy increased with increasing age. The incidence was 0.7%, 6.8%, and 14.3%, in age groups < 10 years old, 10–20 yea
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