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

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

doi:10.1016/j.hbpd.2023.08.011

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

Hepatobiliary Pancreat Dis Int. 2023 Aug 12:S1499-3872(23)00130-3. doi: 10.1016/j.hbpd.2023.08.011. Online ahead of print.

Authors

Jun-Jun Jia¹, Ya-Fen Cheng², Mei-Bao Feng³, Li Liu⁴, Ming-Qi Shuai¹, Xiao-Dong Shen¹, Xiao-Feng Tang¹, Shu-Sen Zheng⁵

Affiliations

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.
⁵ Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China. Electronic address: shusenzheng@zju.edu.cn.

PMID: 37652799
DOI: 10.1016/j.hbpd.2023.08.011

Abstract

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 Affiliated 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.

Keywords: Biliary mucinous cystic neoplasms; High-grade intraepithelial neoplasms; Low-grade intraepithelial neoplasms; Overall survival; Recurrence-free survival.