Cholangiocarcinoma: a review of the literature and future directions in therapy

Abstract

Importance: Cholangiocarcinomas (CCA) are a group of rare cancers with an incidence of about 1.26 per 100,000 people. The disease reflects one of three different subtypes: intrahepatic, perihilar/hilar or distal CCA. The preferred modality of definitive therapy is surgical resection with or without adjuvant therapy. Some efforts to improve survival rates have come in the form of offering neoadjuvant.Objective: The aim of this review article is to identify and discuss different treatment paradigms in intrahepatic, perihilar/hilar or distal CCA.Evidence Review: A literature review was performed to review articles relevant to the topic of neoadjuvant therapy [including the role of liver transplant (LT)], definitive chemoradiotherapy, surgical resection and adjuvant therapy and palliative therapy including both chemotherapy and targeted therapies.Findings: CCAs remain one of the more aggressive adenocarcinoma subtypes. Based on our review, the best treatment option to date is LT. Surgical resection remains an option for a very small portion of patients with this disease type. The role of chemotherapy remains the standard of care for patients in the metastatic setting, however targeted therapy is starting to increase its presence in the literature.Conclusions and Relevance for Reviews: Our treatment of CCA has improved over the years through a variety of advancements in surgical techniques, adjuvant therapy and palliative therapy, however the overall survival remains poor. The field would benefit from future treatments focusing on early detection in order to identify more early stage patients as well as finding new therapeutic targets.

Update on the Applications of Radiomics in Diagnosis, Staging, and Recurrence of Intrahepatic Cholangiocarcinoma

“…In addition, the prediction of the lymph nodes involved can have effects in clinical practice. In fact, early recurrence and involvement of lymph nodes impact the choice of liver transplantation for unresectable ICC despite medical therapy [ 156 , 157 , 158 ]. Precision medicine, and consequently precision oncology, like precision surgery, should be based on these features not immediately visible to the human eye [ 159 , 160 , 161 , 162 ].…”

Current Perspectives in Liver Transplantation for Perihilar Cholangiocarcinoma

“…Despite primary surgical resection, most pCCA patients are not cured of their disease, as disease recurrence is about 80% within the following eight years, with frequent sites of recurrence in the liver, portal lymph nodes, and peritoneum [ 20 ]. For this reason, several centres propose chemoradiotherapy (CRT) not only as neoadjuvant treatment in borderline resectable pCCA (at present, there is no evidence that neoadjuvant CRT is beneficial) [ 21 ], but also as an adjuvant approach to enhance surgical results in cases with lymph nodes positive for disease spread [ 22 ].…”

Complete response of recurrent perihilar cholangiocarcinoma following sintilimab combined with lenvatinib plus S-1: a case report and review of literature

“…The majority of perihilar cholangiocarcinoma patients tend to present at the late stage and can be treated with radical surgical treatment [14,15]. With more accumulated clinical experience and improvements in operative techniques, more aggressive surgical approaches have been introduced in an attempt to ensure negative surgical margins.…”