INTRODUCTION: The aim of this study was to evaluate the results of surgical treatment of intrahepatic cholangiocarcinoma (IHCHCA) in terms of overall survival and disease-free survival, and to analyse and find potential prognostic factors affecting overall survival and disease-free survival. METHODS: Retrospective evaluation was performed of consecutively enrolled patients operated for IHCHCA from January 2005 to January 2022 (17 years) had undergone surgery. During the monitored period, 38 surgical procedures were performed, of which liver resection was done in 25 cases (65.8%). RESULTS: The 5-year survival in the radically resected group was 44%, and the 5-year disease-free survival was 32%. Based on univariate and multivariate analysis, radicality of surgery (p=0.01116) and lymph node involvement (p=0.00576) were assessed as negative prognostic factors for overall survival. Radicality of surgery (p=0.018) and administration of adjuvant chemotherapy (p=0.044) were significant negative prognostic factors affecting disease-free survival. However, they lost their significance in the multivariate analysis. CONCLUSION: Radical surgical resection of the liver remains an essential treatment option for intrahepatic cholangiocarcinoma aimed at achieving a radical procedure with microscopically negative margins. KEY WORDS: cholangiocarcinoma, resection, recurrence, survival.

• Klíčová slova
• Prognosis, cholangiocarcinoma, heart failure, recurrence, resection, survival,
• MeSH
• cholangiokarcinom * chirurgie   patologie   MeSH
• hepatektomie metody   MeSH
• lidé MeSH
• míra přežití MeSH
• nádory žlučových cest * chirurgie   patologie   MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• výsledek terapie MeSH
• žlučové cesty intrahepatální patologie   chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Intrahepatic cholangiocarcinoma (ICC) is the second most common primary liver cancer next to hepatocellular carcinoma (HCC). Despite the significant difference of the therapeutic strategy for both diseases, their histological appearance may be very similar. Thus the correct diagnosis is crucial for treatment choice but is often difficult to achieve. The aim of our study was to evaluate anterior gradient 3 (AGR3) as a new diagnostic marker helping to distinguish between ICC and HCC. AGR3 is a putative transmembrane protein implicated in breast, prostate and ovary tumorigenesis and belongs to the family of protein disulfide isomerases. Since there is little information on how AGR3 is expressed in normal and diseased tissues and what its exact function is, we analyzed its expression pattern in normal liver and tumor tissue of ICC and HCC. The immunohistochemical analysis in normal tissue revealed specific AGR3 expression in intrahepatic bile duct cholangiocytes which was not present in liver hepatocytes. Consequently we analyzed AGR3 expression in 74 representative samples of puncture biopsies, tissue excisions and resection specimens from which 48 samples were diagnosed as HCC and 26 as ICC. Our results showed AGR3 expression negative and weakly positive respectively in hepatocellular carcinomas compared to stronger AGR3 positivity in cholangiocellular carcinomas. AGR3 expression statistically significantly correlated to acid mucopolysaccharide expression and negatively correlated to glypican-3 expression. We conclude that according to receiver operating characteristics (ROC) analysis AGR3 expression is relatively specific for ICC and is potentially linked to mucosecretion, which may indicate potential implication in treatment resistance.

• Klíčová slova
• AGR3, GPC-3, Hepatocellular carcinoma, Immunohistochemistry, Intrahepatic cholangiocarcinoma, Mucopolysaccharides,
• MeSH
• cholangiokarcinom diagnóza   genetika   MeSH
• diferenciální diagnóza MeSH
• dospělí MeSH
• genetické markery MeSH
• glypikany genetika   metabolismus   MeSH
• hepatocelulární karcinom diagnóza   genetika   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádorové biomarkery genetika   metabolismus   MeSH
• nádorové proteiny genetika   metabolismus   MeSH
• nádory jater diagnóza   genetika   MeSH
• nádory žlučových cest MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• transportní proteiny genetika   metabolismus   MeSH
• žlučové cesty intrahepatální metabolismus   patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• AGR3 protein, human MeSH Prohlížeč
• genetické markery MeSH
• glypikany MeSH
• GPC3 protein, human MeSH Prohlížeč
• nádorové biomarkery MeSH
• nádorové proteiny MeSH
• transportní proteiny MeSH

Cholangiocarcinoma represents the second most common primary liver malignancy. Despite it comprises only 3 % of all gastrointestinal malignancies, its incidence has been increasing recently. Cholangiocarcinomas are hepatobiliary cancers with features of cholangiocyte differentiation and from clinical point of view they are classified anatomically as intrahepatic or extrahepatic form. The only curative treatment with aim of long term and disease-free survival is surgery - liver resection or liver transplantation. Current progress in perioperative treatment and increased surgical skills has changed old treatment algorithms and widen number of patients suitable for curative treatment. On the other hand, surgical intervention is connected with not negligible morbidity and mortality. A rigorous knowledge of the disease extent and all prognostic factors is main condition for proper treatment decision.

• Klíčová slova
• Klatskin tumor, cholangiocarcinoma, liver resection, liver transplantation,
• MeSH
• cholangiokarcinom * chirurgie   MeSH
• dospělí MeSH
• hepatektomie MeSH
• lidé MeSH
• nádory jater * chirurgie   MeSH
• žlučové cesty intrahepatální * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Cholangiocarcinoma is a relatively rare malignant tumor arising from the biliary epithelium of the intra- and extrahepatic bile ducts, the gallbladder, and the ampulla of Vater. This review article presents cholangiocarcinoma from the routine histopathological point of view. In addition to an overview of basic morphological, immunohistochemical, and molecular genetic characteristics of cholangiocarcinoma subtypes and precancerous lesions, the article is focused on intraoperative biopsies and on changes in the 8th edition of the TNM classification. Macroscopic and microscopic photo documentation and a review of recent literature are included.

• Klíčová slova
• cholangiocarcinoma, cholangiocellular carcinoma, extrahepatic, intrahepatic, perihilar,
• MeSH
• cholangiokarcinom * patologie   MeSH
• lidé MeSH
• nádory žlučových cest * chirurgie   patologie   MeSH
• patologové MeSH
• žlučové cesty extrahepatické * patologie   MeSH
• žlučové cesty intrahepatální patologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND: The gut microbiota, composed by several species of microorganisms, works to preserve the liver-gut homeostasis and plays an important role during digestion and absorption of nutrients, and in the immune response of the host. In this review, we analyzed the influence of microbiota in patients with cholangiocarcinoma (CCA) who were candidates for elective surgery. METHODS: A literature review was conducted to identify papers that provided empiric evidence to support that the altered microbiota composition (dysbiosis) is related also to CCA development. RESULTS: Bacteria such as Helicobacter pylori, Helicobacter hepaticus, and Opisthorchis viverrini increase the risk of CCA. The most abundant genera were Enterococcus, Streptococcus, Bacteroides, Klebsiella, and Pyramidobacter in CCA's biliary microbiota. Additionally, levels of Bacteroides, Geobacillus, Meiothermus, and Anoxybacillus genera were significantly higher. An enrichment of Bifidobacteriaceae, Enterobacteriaceae, and Enterococcaceae families has also been observed in CCA tumor tissue. Microbiota is related to postoperative outcomes in abdominal surgery. The combination of caloric restriction diets in liver cancer or CCA increases the effect of the chemotherapy treatment. CONCLUSION: The correct use of nutrition for microbiota modulation according to each patient's needs could be a therapeutic tool in combination with elective surgery and chemotherapy to diminish side effects and improve prognosis. Further investigations are needed to fully understand the mechanisms by which they are related.

• Klíčová slova
• Cholangiocarcinoma, Diet, Liver cancer, Microbiota, Surgery,
• MeSH
• cholangiokarcinom * chirurgie   patologie   MeSH
• dysbióza MeSH
• lidé MeSH
• mikrobiota * MeSH
• nádory žlučových cest * chirurgie   MeSH
• opistorchióza * mikrobiologie   MeSH
• žlučové cesty intrahepatální MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND: The aim of the study was to calculate the short-term and long-term outcomes of curative-intent surgery in distal cholangiocarcinoma (DCC) patients to identify potential prognostic factors. PATIENTS AND METHODS: A retrospective cohort study of 32 consecutive DCC patients treated with pancreaticoduodenectomy between 2009-2017. The clinicopathological and histopathological data were evaluated for prognostic factors using the univariable Cox regression analysis. The Overall Survival (OS) was estimated using the Kaplan-Meier analysis. RESULTS: The study comprised a total of 32 patients, with a mean age of 65.8 (± 9.0) years at the time of surgery. R0 resection was achieved in 25 (86.2%) patients, 19 (65.5%) patients received adjuvant oncological therapy. The OS rates at 1, 3 and 5 years were 62.5%, 37.5% and 21.9%, respectively. The 90-day mortality was 3/32 (9.4%) accounting for one-fourth of the first-year mortality rate. The median OS was 28.5 months. The only statistically significant prognostic factor was vascular resection, which was associated with worse OS in the univariable analysis (HR: 3.644; 95%-CI: 1.179-11.216, P=0.025). An age less than 65 years, ASA grade I/II, hospital stay of fewer than 15 days, R0 resection, lymph node ratio less than 0.2 and adjuvant oncological therapy tended to be associated with better OS but without statistically significant relevance. CONCLUSION: The main factor directly influencing the survival of DCC patients is surgical complications. Surgical mortality comprises a significant group of patients, who die in the first year following pancreaticoduodenectomy. Vascular resection is the most important negative prognostic factor for long-term survival.

• Klíčová slova
• cholangiocarcinoma, long-term survival, pancreaticoduodenectomy, prognostic factor,
• MeSH
• cholangiokarcinom * chirurgie   patologie   MeSH
• lidé MeSH
• nádory žlučových cest * chirurgie   patologie   MeSH
• pankreatoduodenektomie MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• senioři MeSH
• výsledek terapie MeSH
• žlučové cesty intrahepatální patologie   MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH

Cholangiocarcinoma is a cancer with very poor prognosis. The only potentially curative approach is surgical resection of tumor. However, the rate of local and distant recurrence after radical surgery is still high. Benefit of adjuvant therapy is not clearly defined, nevertheless patients at high risk of recurrence are indicated to chemotherapy or chemoradiotherapy. Locally advanced, unresectable disease can also be treated with chemotherapy alone, or with her combination with radiotherapy. Required radiation doses are relatively high, therefore it is necessary to use highly conformal radiation therapy. Treatment of metastatic disease is currently based on systemic therapy, combination of gemcitabine and cisplatin as standard of care. Benefit of targeted molecular therapy is not clear at present, but ongoing research in genetic profiling of tumor may help to improve current clinical practice. Patients with cholangiocarcinoma have to be discussed during multidisciplinary team meetings.

• Klíčová slova
• cholangiocarcinoma, drug therapy, radiotherapy adjuvant, targeted therapy,
• MeSH
• chemoradioterapie MeSH
• cholangiokarcinom * terapie   MeSH
• dospělí MeSH
• kombinovaná terapie MeSH
• lidé MeSH
• nádory jater * terapie   MeSH
• nádory žlučových cest * terapie   MeSH
• žlučové cesty intrahepatální * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: ALPPS is found to increase the resectability of primary and secondary liver malignancy at the advanced stage. The aim of the study was to verify the surgical and oncological outcome of ALPPS for intrahepatic cholangiocarcinoma (ICC). METHODS: The study cohort was based on the ALPPS registry with patients from 31 international centers between August 2009 and January 2018. Propensity score matched patients receiving chemotherapy only were selected from the SEER database as controls for the survival analysis. RESULTS: One hundred and two patients undergoing ALPPS were recruited, 99 completed the second stage with median inter-stage duration of 11 days. The median kinetic growth rate was 23 ml/day. R0 resection was achieved in 87 (85%). Initially high rates of morbidity and mortality decreased steadily to a 29% severe complication rate and 7% 90-day morbidity in the last 2 years. Post-hepatectomy liver failure remained the main cause of 90-day mortality. Multivariate analysis revealed insufficient future liver remnant at the stage-2 operation (FLR2) to be the only risk factor for severe complications (OR 2.91, p = 0.02). The propensity score matching analysis showed a superior overall survival in the ALPPS group compared to palliative chemotherapy (median overall survival: 26.4 months vs 14 months; 1-, 2-, and 3-year survival rates: 82.4%, 70.5% and 39.6% vs 51.2%, 21.4% and 11.3%, respectively, p < 0.01). The survival benefit, however, was not confirmed in the subgroup analysis for patients with insufficient FLR2 or multifocal ICC. CONCLUSION: ALPPS showed high efficacy in achieving R0 resections in locally advanced ICC. To get the most oncological benefit from this aggressive surgery, ALPPS would be restricted to patients with single lesions and sufficient FLR2.

• MeSH
• ascites epidemiologie   MeSH
• cholangiokarcinom chirurgie   MeSH
• dospělí MeSH
• hepatektomie metody   MeSH
• infekce chirurgické rány epidemiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• ligace MeSH
• mezinárodní spolupráce MeSH
• míra přežití MeSH
• nádory žlučových cest chirurgie   MeSH
• paliativní péče MeSH
• pooperační komplikace epidemiologie   prevence a kontrola   MeSH
• pooperační krvácení epidemiologie   MeSH
• program SEER MeSH
• proporcionální rizikové modely MeSH
• protinádorové látky terapeutické užití   MeSH
• registrace MeSH
• selhání jater prevence a kontrola   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• tendenční skóre MeSH
• vena portae chirurgie   MeSH
• výsledek terapie MeSH
• žlučové cesty intrahepatální * MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Názvy látek
• protinádorové látky MeSH

Cholangiocarcinoma is a relatively rare, highly fatal neoplasm originating from the biliary epithelium. Its only potentially curative treatment option is a radical surgical resection. The aim of our work was to evaluate the feasibility and the safety of intraoperative ERCP and direct cholangioscopy (SpyGlass) to assess the intraductal border of cholangiocarcinoma proliferation. The study ran from November 2015 to January 2018. The group included patients with histologically verified cholangiocarcinoma and, based on available examinations, the resectability of the tumor was assessed by a multidisciplinary team. In cases of indicated surgical resection we peroperatively performed ERCP with cholangioscopy SpyGlass and "diaphanoscopy" in all patients. The resectability was assessed on the basis of these examinations and the peroperative surgical findings. The resection procedure itself was performed only in 2 out of the total of 14 patients, as other patients were indicated for the implantation of metallic SEMS within the ERCP procedure in the operating room instead. To validate the cholangioscopic findings, we used our own criteria based on both the Monaco and other criteria. We divided the findings according to the presence or absence of ulceration, prominent polyposis, pathological vascularization (4 types), pressure defect with a coagulum in the presence of previous stent implantation, papillomatous changes or discolorations of the mucosa. Out of the total number of 14 patients only two patients were indicated for resection and in both cases R0 resection was achieved. The remaining patients were intraoperatively indicated for palliative implantation of SEMS based on the same unresectable finding during cholangioscopy and laparotomy. We demonstrated the technical feasibility and safety of direct peroperative cholangioscopy. Our results show that direct perioperative cholangioscopy is one of the methods which can contribute to a more accurate determination of tumor spread margins.

• Klíčová slova
• ERCP, cholangiocarcinoma, direct cholangioscopy, intraductal ultrasonography,
• MeSH
• biopsie MeSH
• cholangiokarcinom * diagnóza   MeSH
• dospělí MeSH
• endoskopie trávicího systému MeSH
• lidé MeSH
• nádory žlučových cest * diagnóza   MeSH
• pilotní projekty MeSH
• proliferace buněk MeSH
• žlučové cesty intrahepatální * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

AIMS AND BACKGROUND: Conventional radiotherapy in inoperable cholangiocarcinoma is limited by radiotolerance of the surrounding tissues. The aim of our dosimetric study was an evaluation of intensity-modulated radiotherapy in the treatment of inoperable bile duct carcinoma. METHODS: Four patients with inoperable cholangiocarcinoma treated by self-expandable stent placed to the biliary tree and radiotherapy were studied. The rotational technique, conformal 3D BOX technique and intensity-modulated radiotherapy plan were compared. Dose volume histograms and the normal tissue complication probability concept were used for comparison. The stent was used for target motion verification. RESULTS: The intensity-modulated radiotherapy plans showed favorable dose distribution in planning target volume and remarkable sparing of organs at risk. CONCLUSIONS: The intensity-modulated radiotherapy technique in bile duct carcinomas deserves further research and clinical evaluation.

• MeSH
• celková dávka radioterapie MeSH
• cholangiokarcinom radioterapie   terapie   MeSH
• kombinovaná terapie MeSH
• lidé MeSH
• nádory žlučových cest radioterapie   terapie   MeSH
• paliativní péče MeSH
• počítačová simulace MeSH
• radioterapie s modulovanou intenzitou metody   MeSH
• stenty MeSH
• žlučové cesty extrahepatické * MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• srovnávací studie MeSH