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World J Hepatol. Nov 27, 2020; 12(11): 897-907
Published online Nov 27, 2020. doi: 10.4254/wjh.v12.i11.897
Paraneoplastic syndromes in cholangiocarcinoma
Saad Ur Rahman, Muhammad Khawar Sana, Zabreen Tahir, Aamir Ali, Pir Ahmad Shah
Saad Ur Rahman, Muhammad Khawar Sana, Department of Medicine, King Edward Medical University, Lahore 54000, Punjab, Pakistan
Zabreen Tahir, Department of Neurology, Brigham and Women’s Hospital, Boston, MA 02115, United States
Aamir Ali, Department of Radiology, Beth Israel Deaconess Medical Center, Boston, MA 02215, United States
Pir Ahmad Shah, Department of Medicine, University of Texas Health Science Center, San Antonio, TX 78229, United States
Author contributions: Shah PA and Tahir Z designed the study. Rahman SU and Sana MK did database screening and data extraction. Tahir Z, Shah PA, Rahman SU, Ali A, and Sana MK did the literature review, analyzed the data, and wrote the manuscript. Rahman SU, Sana MK, Ali A, Tahir Z, and Shah PA reviewed the paper for final submission.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Pir Ahmad Shah, MD, Internal Medicine Resident, Department of Medicine, University of Texas Health Science Center, 7703 Floyd Curl Dr, San Antonio, TX 78229, United States. pirashah2020@gmail.com
Received: June 16, 2020
Peer-review started: June 16, 2020
First decision: July 30, 2020
Revised: August 13, 2020
Accepted: September 8, 2020
Article in press: September 8, 2020
Published online: November 27, 2020
Processing time: 161 Days and 7.4 Hours
Abstract

Paraneoplastic syndromes are the symptoms or signs which result from damage to tissues that are distant from the site of malignancy, due to complex interactions between the body’s immune system and malignant neoplasm. Cholangiocarcinoma (CCA) is an aggressive epithelial malignancy of hepatobiliary tree and it is found to be associated with various paraneoplastic syndromes. These syndromes can present as dermatological, neurological, renal, hematological, or multi-systemic manifestations. Clinical suspicion and timely recognition of these syndromes can lead to early diagnosis of covert malignancies like CCA. The management plan remains the removal of the underlying cause which in this case is CCA.

Keywords: Cholangiocarcinoma; Paraneoplastic syndrome; Malignancy; Immune system; Biliary tree; Multi-organ

Core Tip: Various case reports have been published focusing on single paraneoplastic syndromes associated with cholangiocarcinoma (CCA) but none of the studies has reported these manifestations collectively. This review summarizes different paraneoplastic syndromes which are associated with CCA to give a better idea about how it affects various organ systems.