cholangiocarcinoma
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Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation and progressive fibrosis of the biliary tree, leading to significant liver function impairment over time. There is a strong association with inflammatory bowel diseases (IBD), together representing a distinct and complex medical condition. Patients with PSC-IBD face a heightened risk of various cancers, particularly colorectal carcinoma (CRC) and cholangiocarcinoma (CCA) as the most common types. In this review, we aim to characterize the distinctive features of PSC-IBD-associated carcinomas. Cancer pathogenesis in PSC-IBD is shaped by various factors including dysregulated bile acid metabolism, gut dysbiosis, and unique immune responses. PSC-IBD-associated CRC is often right-sided and warrants vigilant monitoring due to its higher incidence and unique morphological features compared to CRC arising in the terrain of IBD alone. CCA shares substantial genetic similarities with extrahepatic CCA and poses diagnostic challenges since it is frequently detected at advanced stages due to symptom overlap with PSC. Besides, reliable predictive biomarkers for targeted therapy remain largely unexplored. The distinct molecular, genetic, and histopathological profiles of CRC and CCA in PSC-IBD underscore the complexity of these malignancies and highlight the need for continued research to develop precise therapeutic strategies.
- Klíčová slova
- Cholangiocarcinoma, Colorectal carcinoma, Crohn’s disease, Inflammatory bowel disease, Primary sclerosing cholangitis, Ulcerative colitis,
- MeSH
- cholangiokarcinom * patologie etiologie genetika MeSH
- idiopatické střevní záněty * komplikace patologie MeSH
- kolorektální nádory * patologie etiologie genetika MeSH
- lidé MeSH
- nádorové biomarkery genetika MeSH
- nádory žlučových cest * patologie etiologie genetika MeSH
- sklerozující cholangitida * komplikace patologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Názvy látek
- nádorové biomarkery MeSH
Biliary drainage is then one of the necessary procedures to help patients suffering from icterus to reduce serum bilirubin levels and relieve symptoms. The aim of this study was identifying risk factors for survival in patients with cholangiocarcinoma (CCA) treated with percutaneous transhepatic biliary drainage (PTBD) and to develop a simple scoring system predicting survival from PTBD insertion. This single-centre retrospective study included 175 consecutive patients undergoing PTBD for extrahepatic CCA (perihilar and distal). Prognostic factors affecting survival of patients with CCA treated with PTBD were analysed. A multivariate analysis showed that mass forming tumor with mass larger than 5 cm and presence of metastasis at the time of PTBD served as a negative prognostic factor (p = 0.002), better survival was associated with lower preprocedural bilirubin and lower CRP (p = 0.003). Multivariate analysis identified two significant risk factors for 3-month mortality: mass-forming tumors and bilirubin levels exceeding 185 µmol/L. A simple scoring system was developed to predict 3-month mortality after PTBD in patients with advanced CCA, demonstrating 86.3% negative predictive value and 43.2% positive predictive value.
- Klíčová slova
- Biliary malignancy, Biliary stenosis, Metal stent, Percutaneous transhepatic biliary drainage, Prognostic factors, Scoring system,
- MeSH
- bilirubin krev MeSH
- cholangiokarcinom * mortalita patologie terapie chirurgie MeSH
- dospělí MeSH
- drenáž * metody MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory žlučových cest * mortalita patologie terapie chirurgie MeSH
- prognóza MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- senioři nad 80 let MeSH
- senioři 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
- práce podpořená grantem MeSH
- Názvy látek
- bilirubin MeSH
BACKGROUND: Extended liver resection is the only treatment option for perihilar cholangiocarcinoma (pCCA). Bile salts and the gut hormone FGF19, both promoters of liver regeneration (LR), have not been investigated in patients undergoing resection for pCCA. We aimed to evaluate the bile salt-FGF19 axis perioperatively in pCCA and study its effects on LR. METHODS: Plasma bile salts, FGF19, and C4 (bile salt synthesis marker) were assessed in patients with pCCA and controls (colorectal liver metastases), before and after resection on postoperative days (PODs) 1, 3, and 7. Hepatic bile salts were determined in intraoperative liver biopsies. RESULTS: Partial liver resection in pCCA elicited a sharp decline in bile salt and FGF19 plasma levels on POD 1 and remained low thereafter, unlike in controls, where bile salts rose gradually. Preoperatively, suppressed C4 in pCCA normalized postoperatively to levels similar to those in the controls. The remnant liver volume and postoperative bilirubin levels were negatively associated with postoperative C4 levels. Furthermore, patients who developed postoperative liver failure had nearly undetectable C4 levels on POD 7. Hepatic bile salts strongly predicted hyperbilirubinemia on POD 7 in both groups. Finally, postoperative bile salt levels on day 7 were an independent predictor of LR. CONCLUSIONS: Partial liver resection alters the bile salt-FGF19 axis, but its derailment is unrelated to LR in pCCA. Postoperative monitoring of circulating bile salts and their production may be useful for monitoring LR.
- MeSH
- fibroblastové růstové faktory * krev MeSH
- hepatektomie * MeSH
- játra metabolismus chirurgie MeSH
- Klatskinův nádor * chirurgie patologie krev MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory žlučových cest * chirurgie patologie krev MeSH
- regenerace jater * fyziologie MeSH
- senioři MeSH
- studie případů a kontrol MeSH
- žlučové kyseliny a soli * krev metabolismus MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Názvy látek
- FGF19 protein, human MeSH Prohlížeč
- fibroblastové růstové faktory * MeSH
- žlučové kyseliny a soli * MeSH
BACKGROUND: Endoluminal radiofrequency ablation (RFA) has been promoted as palliative treatment for patients with cholangiocarcinoma (CCA) and pancreatic ductal adenocarcinoma (PDAC) in order to improve biliary drainage and eventually prolong survival. No high level evidence is, however, available on this technique. DESIGN: In this randomised controlled study, we compared endoluminal RFA plus stenting with stenting alone (control group) in patients with malignant biliary obstruction; metal stents were primarily placed. Primary outcome was overall survival; secondary outcomes were stent patency, quality of life and adverse events. In a superiority design, survival was assumed to be doubled by RFA as compared with 6.4 months in the control group (n=280). RESULTS: A total of 161 patients (male:female 90:71, mean age 71±9 years) were randomised before recruitment was terminated for futility after an interim analysis. Eighty-five patients had CCA (73 hilar, 12 distal) and 76 had pancreatic cancer. There was no difference in survival in both subgroups: for patients with CCA, median survival was 10.5 months (95% CI 6.7 to 18.3) in the RFA group vs 10.6 months (95% CI 9.0 to 24.8), p=0.58)) in the control group. In the subgroup with pancreatic cancer, median survival was 6.4 months (95% CI 4.3 to 9.7) for the RFA vs 7.7 months (95% CI 5.6 to 11.3), p=0.73) for the control group. No benefit was seen in the RFA group with regard to stent patency (at 12 months 40% vs 36% in CCA and 66% vs 65% in PDAC), and quality of life was unchanged by either treatment and comparable between the groups. Adverse events occurred in seven patients in each groups. CONCLUSION: A combination of endoluminal RFA and stenting was not superior to stenting alone in prolonging survival or improving stent patency in patients with malignant biliary obstruction. TRIAL REGISTRATION NUMBER: NCT03166436.
- Klíčová slova
- biliary obstruction, cholangiocarcinoma, pancreatic cancer, stents,
- MeSH
- cholangiokarcinom * MeSH
- cholestáza * etiologie chirurgie MeSH
- katetrizační ablace * škodlivé účinky metody MeSH
- kvalita života MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory slinivky břišní * komplikace chirurgie MeSH
- nádory žlučových cest * komplikace chirurgie MeSH
- radiofrekvenční ablace * MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- stenty škodlivé účinky MeSH
- výsledek terapie MeSH
- žlučové cesty intrahepatální chirurgie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- randomizované kontrolované studie MeSH
The liver fluke Opisthorchis viverrini (Poirier, 1886) (Digenea) secretes extracellular vesicles (EVs) bearing CD63-like tetraspanins on their surface. Fluke EVs are actively internalised by host cholangiocytes in the bile ducts, where they drive pathology and promote neoplasia through induction of cellular proliferation and secretion of inflammatory cytokines. We investigated the effects of tetraspanins of the CD63 superfamily by co-culturing recombinant forms of the large extracellular loop (LEL) of O. viverrini tetraspanin-2 (rLEL-Ov-TSP-2) and tetraspanin-3 (rLEL-Ov-TSP-3) with non-cancerous human bile duct (H69) and cholangiocarcinoma (CCA, M213) cell lines. The results showed that cell lines co-cultured with excretory/secretory products from adult O. viverrini (Ov-ES) underwent significantly increased cell proliferation at 48 hours but not 24 hours compared to untreated control cells (P < 0.05), whereas rLEL-Ov-TSP-3 co-culture resulted in significantly increased cell proliferation at both 24 hours (P < 0.05) and 48 hours (P < 0.01) time points. In like fashion, H69 cholangiocytes co-cultured with both Ov-ES and rLEL-Ov-TSP-3 underwent significantly elevated Il-6 and Il-8 gene expression for at least one of the time points assessed. Finally, both rLEL-Ov-TSP-2 and rLEL-Ov-TSP-3 significantly enhanced migration of both M213 and H69 cell lines. These findings indicated that O. viverrini CD63 family tetraspanins can promote a cancerous microenvironment by enhancing innate immune responses and migration of biliary epithelial cells.
- Klíčová slova
- CD63-like tetraspanins, cholangiocarcinoma, extracellular vesicle (EVs), host-parasite interaction,
- MeSH
- buněčné linie MeSH
- cytokiny MeSH
- dospělí MeSH
- epitelové buňky MeSH
- Fasciola hepatica * MeSH
- lidé MeSH
- Opisthorchis * MeSH
- zvířata MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- cytokiny MeSH
BACKGROUND AND AIMS: Despite the undeniable ongoing development of cross-sectional imaging methods, not all focal liver lesions (FLLs) have a typical pattern. An image-guided biopsy using a percutaneous approach might safely provide a final histological diagnosis of the FLLs. We aimed to evaluate the accuracy, efficiency, complication rate, technical features, and relationships between the followed parameters of computed tomography (CT)-guided percutaneous biopsies of FLLs using a retrospective approach. METHODS: 303 percutaneous biopsy procedures in 295 patients were carried out in patients with suspected or indeterminate FLLs over a 10-year period. The median size of the tumors was 44 mm (15 - 144 mm). Median age of patients was 67 years (25 to 87 years). Skin-to-lesion distance was variable, from 30 mm to 138 mm (median length 59 mm). In 200 procedures (66%) malignant disease was known from the patients´ clinical history. RESULTS: In 288 biopsies (95%) the results were true positive or true negative; 15 procedures (4.95%) resulted in a histologically false negative and had to be confirmed using other approaches. Metastatic disease to hepatic parenchyma of various origins was the most frequent histological diagnosis (55.4%). Cholangiocarcinoma was the most common individual result (13.5%). In total 14 complications (4.6%) were confirmed, 4 of which were severe haemorrhages that needed angiographic treatment and in one case surgical revision. The mortality rate in our group was 0.3%. A statistically significant relationship between lesion size and diagnostic accuracy (p < 0.01) was revealed. The use of a 16 G needle calibre and at least two samples were suitable for hypo- and hypervascular lesions without a significant increase in the complication rate. CONCLUSIONS: Core needle biopsy using a percutaneous approach and a CT-guidance performed on patients with indetermined FLLs had a high overall accuracy in determining the final histological diagnosis including subtyping. Concurrently, the complication incidence was low.
Hemobilia is an unusual type of gastrointestinal bleeding most frequently due to iatrogenic injury, trauma, or neoplasia. Acute cholecystitis as a cause of hemobilia is rare. We present the case study of a patient with bleeding from eroded gallbladder mucosa in the setting of severe calculous cholecystitis. The hemorrhagic episode was preceded by acute ERCP due to obstructive icterus with extraction of the calculi, followed by the development of severe acute pancreatitis. These factors initially misled the diagnosis. The bleeding was not hemodynamically important and routine diagnostic methods did not reveal its exact source. Direct choledochoscopy (SpyGlassTM) proved to be helpful in determining the right diagnosis, as it ruled out any injury or tumor in the main bile ducts and considerably supported the assumption of intrabladder bleeding. Surgical revision confirmed the cause, and subsequent cholecystectomy solved the whole problem.
- Klíčová slova
- cholangiocarcinoma, cholangioscopy, choledochoscopy, endoscopic intervention, hemobilia, hemorrhagic cholecystitis,
- MeSH
- akutní cholecystitida * diagnóza diagnostické zobrazování MeSH
- akutní nemoc MeSH
- cholecystitida * komplikace chirurgie MeSH
- gastrointestinální krvácení etiologie MeSH
- hemobilie * komplikace diagnóza MeSH
- lidé MeSH
- pankreatitida * komplikace MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články 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
Many environmental risk factors for hepatobiliary cancers are known but whether they are associated with specific cancer types is unclear. We present here a novel approach of assessing standardized incidence ratios (SIRs) of previously diagnosed comorbidities for hepatocellular carcinoma (HCC), gallbladder cancer (GBC), cholangiocarcinoma (CCA) and ampullary cancer. The 13 comorbidities included alcohol and nonalcohol related liver disease, chronic obstructive pulmonary disease, gallstone disease, viral and other kinds of hepatitis, infection of bile ducts, hepatic and other autoimmune diseases, obesity and diabetes. Patients were identified from the Swedish Inpatient Register from 1987 to 2018, and their cancers were followed from 1997 onwards. SIRs for HCC were 80 to 100 in men and women diagnosed with hepatitis C virus and they were also >10 in patients diagnosed with hepatitis B virus, other kind of hepatitis, hepatic autoimmune disease and nonalcohol related liver disease. Many of these risks, as well as alcohol related liver disease, were either specific to HCC or were shared with intrahepatic CCA. For GBC, CCA and ampullary cancer infection of bile ducts was the main risk factor. Gallstone disease, nonhepatic autoimmune diseases and diabetes were associated with all hepatobiliary cancers. The limitations of the study include inability to cover some rare risk factors and limited follow-up time. Many of the considered comorbidities are characterized by chronic inflammation and/or overt immune disturbance in autoimmune diseases. The results suggest that local chronic inflammation and a related immune disturbance is the carcinogenic trigger for all these cancers.
- Klíčová slova
- alcohol, comorbidity, familial risk, hepatocellular carcinoma, risk factor, smoking,
- MeSH
- ampulla Vateri * patologie MeSH
- autoimunitní nemoci * MeSH
- cholangiokarcinom * epidemiologie etiologie diagnóza MeSH
- cholelitiáza * komplikace patologie MeSH
- hepatocelulární karcinom * patologie MeSH
- lidé MeSH
- nádory ductus choledochus * komplikace patologie MeSH
- nádory jater * epidemiologie etiologie patologie MeSH
- nádory žlučníku * etiologie komplikace MeSH
- nádory žlučových cest * epidemiologie etiologie patologie MeSH
- zánět patologie MeSH
- žlučové cesty intrahepatální patologie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
