ABSTRACT
Introduction
Intrahepatic cholangiocarcinoma (ICC) incidence continues to rise worldwide, and overall survival remains poor. Complete surgical resection remains the only opportunity for cure in patients with ICC yet only one-third of patients present with resectable disease.
Areas covered
While the low incidence rate of ICC hinders accrual of patients to large, randomized control trials, larger database and long-term institutional studies provide evidence to guide surgical management of ICC. These studies demonstrate feasibility, safety, and efficacy of aggressive surgical management in appropriately selected patients with ICC. Recent advances in the management of ICC, with a focus on surgical considerations, are reviewed.
Expert opinion
Historically, little progress has been made in the management of ICC with stagnant mortality rates and poor long-term outcomes. However, regionalization of care to centers with experienced multidisciplinary teams, advances in minimally invasive surgical techniques, discovery and development of targeted and immunotherapy agents, and combination locoregional and systemic therapies offer signs of progress in the management of ICC.
Article highlights
Surgical resection remains the only chance for long-term survival in patients with ICC; standard of care surgical management includes hepatectomy with negative margins and sufficient functional liver remnant
In select patients managed at high-volume centers by experienced surgeons and multidisciplinary care teams, major vascular resection with reconstruction to achieve negative margins is warranted and can be achieved with acceptable morbidity and mortality
Lymphadenectomy of the periportal lymph nodes is recommended; however, broad adoption of this practice is incomplete
When performed by qualified and experienced surgeons, minimally invasive surgical approaches provide acceptable oncologic and morbidity outcomes
Orthotopic liver transplantation should be limited to highly selected patients treated at specialized centers on protocol as outcomes are inferior to liver transplant outcomes for other indications
When combined with systemic chemotherapy, locoregional therapies may expand the cohort of resectable patients and improve outcomes for patients with ICC
IDH1 mutations and FGFR2 fusions are promising molecular targets for ICC therapy
Declaration of interest
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
Reviewer disclosures
Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.