Intrahepatic cholangiocarcinomas represent rare and aggressive malignancies developing from the second order bile ducts to the smaller biliary branches. The aim of this narrative review is to discuss about the main diagnostic and therapeutic challenges in order to help medical and surgical oncologists to gain familiarity in regard to this subject. Articles discussing about epidemiology, histology, diagnostic, perioperative management and surgery which were published from January 2000 to September 2023 included in Cochrane Library, PubMed, Embase, MedLine, Web of Science, Elsevier, Google Scholar databases were reviewed. Articles reviewed in the current paper came to demonstrate that the main problem in such cases is related to the fact that most cases remain asymptomatic for a long period of time and therefore are diagnosed in advanced stages of the disease when curative procedures are feasible after performing extended visceral sacrifice or even worse, are no longer possible; however, the most efficient therapeutic strategy in order to improve the long term outcomes remains radical surgery. In this respect, attention was focused on improving the accuracy of the diagnostic tools and on identifying non-surgical therapeutic options which might increase the chances of achieving complete resection. Intrahepatic cholangiocarcinoma represent rare aggressive tumors with poor outcomes especially if radical surgery is not feasible.
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