Diagnosis and treatment of biliary mucinous cystic neoplasms: A single-center experience  

作　　者：Jun-Jun Jia Ya-Fen Cheng Mei-Bao Feng Li Liu Ming-Qi Shuai Xiao-Dong Shen Xiao-Feng Tang Shu-Sen Zheng 

机构地区：[1]Division of Hepatobiliary and Pancreatic Surgery,Department of Surgery,the First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China [2]Department of International Healthcare Center,the First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China [3]Department of Pathology,the First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China [4]Department of Library,the First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China

出　　处：《Hepatobiliary & Pancreatic Diseases International》2024年第5期495-501,共7页国际肝胆胰疾病杂志（英文版）

基　　金：supported by grants from Zhejiang Natural Science Foundation (GF21H030024);the General Scientific Re-search Project of Zhejiang Provincial Department of Education (Y202146219);the Postgraduate Education Research Project of Zhejiang University (20220326)。

摘　　要：Background: Biliary mucinous cystic neoplasms(BMCNs) are rare hepatobiliary cystic tumors, which can be divided into noninvasive and invasive types. This study aimed to investigate the diagnosis, treatment, and prognosis of BMCNs in a large single center. Methods: We analyzed 49 patients with BMCNs confirmed by postoperative pathology at the First Afflliated Hospital, Zhejiang University School of Medicine between January 2007 and December 2021. Results: Among the 49 patients, 37 were female(75.5%), and the average age was 57.04 years. Common symptoms included abdominal discomfort, jaundice and fever, while 22 patients(44.9%) had no symptoms. Serum carbohydrate antigen(CA) 19-9 and CA125 concentrations were elevated in 34.8% and 19.6% of patients, respectively. Forty-eight patients had tumors in the intrahepatic bile ducts and only one had a tumor in the extrahepatic bile duct. Forty-eight patients with noninvasive intrahepatic BMCNs were further analyzed in terms of pathological features: 34(70.8%) had low-grade intraepithelial neoplasms(LGINs), and 14(29.2%) had high-grade intraepithelial neoplasms(HGINs). The potential immunohistochemical markers of BMCNs were cytokeratin(CK) 19, CK7, estrogen receptor and progesterone receptor. Follow-up data for 37 patients with intrahepatic BMCNs were obtained. The median overall survival(OS) of BMCNs was not reached. The longest survival time was 137 months.The 5-and 10-year OS rates were 100% and 85.4%, respectively. The 5-and 10-year recurrence-free survival(RFS) rates were 93.9% and 80.2%, respectively. Conclusions: BMCNs are rare cystic neoplasms that commonly occur in middle-aged females. BMCNs can only be diagnosed and classified by postoperative pathology, as there are no specific clinical presentations, serological indicators or imaging modalities for preoperative diagnosis. Complete surgical resection is necessary for BMCNs, and the postoperative prognosis is favorable.

关 键 词：Biliary mucinous cystic neoplasms Low-grade intraepithelial neoplasms High-grade intraepithelial neoplasms Overall survival Recurrence-free survival 

分 类 号：R735.8[医药卫生—肿瘤]