Practical review for diagnosis and clinical management of perihilar cholangiocarcinoma

Abstract

Cholangiocarcinoma (CCC) is the most aggressive malignant tumor of the biliary tract. Perihilar CCC (pCCC) is the most common CCC and is burdened by a complicated diagnostic iter and its anatomical location makes surgical approach burden by poor results. Besides its clinical presentation, a multimodal diagnostic approach should be carried on by a tertiary specialized center to avoid miss-diagnosis. Preoperative staging must consider the extent of liver resection to avoid post-surgical hepatic failure. During staging iter, magnetic resonance can obtain satisfactory cholangiographic images, while invasive techniques should be used if bile duct samples are needed. Consistently, to improve diagnostic potential, bile duct drainage is not necessary in jaundice, while it is indicated in refractory cholangitis or when liver hypertrophy is needed. Once resecability criteria are identified, the extent of liver resection is secondary to the longitudinal spread of CCC. While in the past type IV pCCC was not considered resectable, some authors reported good results after their treatment. Conversely, in selected unresectable cases, liver transplantation could be a valuable option. Adjuvant chemotherapy is the standard of care for resected patients, while neoadjuvant approach has growing evidences. If curative resection is not achieved, radiotherapy can be added to chemotherapy. This multistep curative iter must be carried on in specialized centers. Hence, the aim of this review is to highlight the main steps and pitfalls of the diagnostic and therapeutic approach to pCCC with a peculiar attention to type IV pCCC.

Hepatopancreatoduodenectomy for locally advanced perihilar cholangiocarcinoma: A case report and a plea not to underestimate surgical resectability

“…Complete resection is the most effective treatment for this kind of malignancy, as the role of adjuvant chemotherapy and radiotherapy remains controversial in medical literature: specifically, the studies that successfully demonstrated advantages of adjuvant chemoradiation therapy evidenced a survival benefit of only a few months. Resectability of perihilar cholangiocarcinomas remains limited (<50 %) due to late presentation, and should be accurately evaluated accounting tumour extension, patient status and liver function [8] . The complexity surgical treatment and clinical management mandates surgical eligibility to be assessed in high-volume and expertise centres, which are characterized by lower failure-to-rescue from surgical complications and consequently lower in-hospital and 3-months mortality [9] .…”

Liver transplantation in malignant disease

“…Radical resection is the state-of-the art therapy and provides a 5-year OS of between 20% and 50% in cases with R0 resection[ 97 , 98 ]. However, a significant proportion of patients cannot undergo resection either because of distant metastases, a locally advanced tumor, quality of liver parenchyma, or underlying liver disease[ 99 ]. Hence, liver transplantation might be the only remaining curative option for those patients.…”

Personalized Endoscopy in Complex Malignant Hilar Biliary Strictures

“…The patient with HBO generally presents cholestasis and obstructive jaundice, in this setting the first-line imaging technique is classically an abdominal ultrasound (US). Although it is a useful tool in the initial management, US is an operator-dependent technique and is burdened by low sensitivity and specificity [ 6 ]. Multidetector computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance cholangiopancreatography (MRCP) are generally required to perform an accurate diagnosis.…”

“…Endoscopic Retrograde Cholangio-Pancreatography (ERCP) is nowadays considered a therapeutic procedure, however, during ERCP several diagnostic tools can be used—brush cytology, endobiliary biopsy, and cholangioscopy. Brush cytology is an easy procedure, routinely performed in suspicious biliary strictures, characterized by low sensitivity (ranges 20–40%) and high specificity (ranges 92–100%) [ 6 ]. The sensibility can be improved by performing multiple brushings during the same ERCP and performing fluorescence in situ hybridization (FISH) analysis on the sample [ 9 , 10 ].…”