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.
- MeSH
- Cholangiocarcinoma * pathology etiology genetics MeSH
- Inflammatory Bowel Diseases * complications pathology MeSH
- Colorectal Neoplasms * pathology etiology genetics MeSH
- Humans MeSH
- Biomarkers, Tumor genetics MeSH
- Bile Duct Neoplasms * pathology etiology genetics MeSH
- Cholangitis, Sclerosing * complications pathology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Review 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.
- MeSH
- Ampulla of Vater * pathology MeSH
- Autoimmune Diseases * MeSH
- Cholangiocarcinoma * epidemiology etiology diagnosis MeSH
- Cholelithiasis * complications pathology MeSH
- Carcinoma, Hepatocellular * pathology MeSH
- Humans MeSH
- Common Bile Duct Neoplasms * complications pathology MeSH
- Liver Neoplasms * epidemiology etiology pathology MeSH
- Gallbladder Neoplasms * etiology complications MeSH
- Bile Duct Neoplasms * epidemiology etiology pathology MeSH
- Inflammation pathology MeSH
- Bile Ducts, Intrahepatic pathology MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Infekce virem hepatitidy B (HBV) zůstává celosvětovým zdravotním problémem, je s ní spojena významná morbidita a mortalita. Globálně je odhadováno více než 240 milionů jedinců s chronickou HBV infekcí. U neléčených vede nemoc v 15–40 % k rozvoji cirhózy (CH), která může progredovat do jaterního selhání nebo hepatocelulárního karcinomu (HCC) (4). Epidemiologie tohoto onemocnění se změnila zavedením očkování. Ovlivňují ji však i další faktory, např. migrace. Vakcinace se v České republice provádí v rámci pravidelného (od roku 2001), zvláštního a doporučeného očkování. Autoři předkládají kazuistiku mladého muže s chronickou HBV infekcí, u kterého se po 26 letech jejího trvání rozvinul cholangiocelulární karcinom (CC).
Hepatitis B virus (HBV) infection remains a global public health problem with significant morbidity and mortality. More than 240 million individuals worldwide are infected with HBV. Among the untreated, 15 to 40 % progress to cirrhosis (CH), which may lead to liver failure and liver cancer. Epidemiology of this disease has changed due to several factors including vaccination policies and migration. In the Czech Republic vaccination is carried out within regular (from 2001), specific and recommended vaccination. Authors describe a case report of a young man with chronic HBV infection and cholangiocarcinoma (CC) which developed after 26 years long infection.
- MeSH
- Cholangiocarcinoma * diagnosis etiology therapy MeSH
- Hepatitis B, Chronic * diagnosis drug therapy complications prevention & control MeSH
- Adult MeSH
- Humans MeSH
- Magnetic Resonance Imaging methods MeSH
- Death MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Male MeSH
- Publication type
- Case Reports MeSH
PSC je chronické cholestatické ochorenie pečene charakterizované progresívnym zápalom a fibrózou žlčovodov, vyúsťujúce do biliárnej cirhózy a s vysokým rizikom vzniku cholangiokarcinómu. Jeho etiológia nie je známa a okrem transplantácie pečene nejestvuje ani kuratívna liečba. Zápalové zmeny extrahepatálnych žlčovodov vedú k tvorbe takmer kompletných stenóz s akútnym zhoršením pečeňových funkcii. Takéto lézie sa nazývajú dominantné striktúry a postihujú 15-20 % PSC pacientov. Endoskopická liečba sa zameriava najmä na striktúry pravého a ľavého hepatiku ako aj spoločného hepatiku, resp. hepatocholedochu. Endoskopická liečba ponúka balónikovú dilatáciu dominantných stenóz alebo ich dilatáciu búžiami a zakladanie duodenobiliárnych drenáží, prípadne kombináciu oboch týchto metód. Ktorýkoľvek z týchto prístupov vedie k predĺženiu prežívania PSC pacientov, aj keď dosiaľ nie je arbitrážne stanovená najvhodnejšia endoskopická liečebná metóda na riešenie dominantných stenóz. Väčšina centier uprednostňuje ich balónikovú dilatáciu kvôli nižšiemu výskytu komplikácii (hnisavá cholangitída, sepsa). Prítomnosť distálne lokalizovanej dominantnej stenózy, vysoká hyperbilirubinémia a včasná endoskopická liečba dominantných stenóz sú nezávislými prediktívnymi faktormi úspešného efektu terapie PSC.
PSC is a chronic cholestatic liver disease characterized by progressive inflammation and fibrosis of the bile ducts, resulting in biliary cirrhosis associated with a high risk of cholangiocarcinoma. PSC is a disease with unknown aetiology and without any further curative treatment options apart from liver transplantation. Inflammatory alterations in PSC can lead to almost complete stenosis of the extrahepatic biliary tree and cause acute deterioration of liver function. Such lesions are termed as dominant biliary stenoses which occur in 15-20 % of PSC patients. Endoscopic treatment is concerned to strictures localised in the common bile duct and main hepatic ducts. Current endoscopic therapy consists of either bougienage or balloon dilation of strictures with or without concomitant placement of endoprosthesis. Endoscopic treatment has a beneficial effect on survival of PSC patients regardless of no general recommendation for best endoscopic approach to dominant strictures. Although baloon dilation has the disadvantage of early restenosis, most centers have preferred endoscopic balloon dilations because of lower incidence of complications like suppurative cholangitis and sepsis. Presence of a distal dominant stricture, endoscopic therapy and high serum bilirubin are the independent predictors of a successful clinical outcome.
- MeSH
- Liver Cirrhosis, Biliary diagnosis etiology MeSH
- Cholangiocarcinoma diagnosis etiology MeSH
- Cholestasis diagnosis etiology therapy MeSH
- Diagnostic Techniques, Digestive System utilization MeSH
- Endoscopy, Digestive System methods utilization MeSH
- Drug Therapy methods utilization MeSH
- Humans MeSH
- Evidence-Based Medicine MeSH
- Cholangitis, Sclerosing diagnosis etiology therapy MeSH
- Liver Transplantation utilization MeSH
- Check Tag
- Humans MeSH
- MeSH
- Cholangiocarcinoma diagnosis etiology pathology MeSH
- Hemangiosarcoma diagnosis etiology MeSH
- Hepatoblastoma diagnosis classification pathology MeSH
- Adenoma, Liver Cell diagnosis therapy MeSH
- Carcinoma, Hepatocellular diagnosis etiology classification pathology MeSH
- Hemangioma, Cavernous diagnosis MeSH
- Humans MeSH
- Liver Neoplasms * diagnosis etiology classification pathology MeSH
- Liver Diseases diagnosis etiology classification pathology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Lecture MeSH
Cíl: Zhodnoceni efektu brachyterapie (Iridium - 192) v kombinaci se zavedením kovového stentu V prospektivní randomizované studii. Materiál a metodika: Celkem bylo ošetřeno 76 nemocných. Etiologie stenózy byla u všech ověřena histologický. 38 pacientů s inoperabilní maligní stenózou žlučových cest (skupina A) bylo léčeno perkutánní implantací stentu (Wallstent, spirální Z stent) a následnou brachyterapií (Ir-192, průměrná dávka 30 Gy). 38 nemocných (skupina B) mělo zavedeno jen Wallstent bez následného ozařování. Výsledky: Průměrná délka života nemocných (skupina A) byla 291,2, resp. (skupina B) 195,1 dnů. Ze statistického hodnocení jsme vyřadili pacienty, kteří zemřeli do jednoho měsíce. Závěr: Kombinace perkutánního zavedení stentu a následné rádioterapie statisticky významně prodlužuje délku života u nemocných s maligní stenózou žlučových cest léčenou zavedením kovového stentu. Nejvýraznější rozdíl jsme zaznamenali u nemocných s cholangiokarcinomem (339,8 A versus 298 B).
Objective: Evaluation of the effect of brachytherapy (Iridium-192) combined with insertion of a metal stent in a prospective randomized study. Material and methods: A total of 76 patients were treated. The etiology of stenosis was in all confirmed by histological examination. 38 patients with an inoperable malignant stenosis of the biliary pathways (group A) was treated by percutaneous implantation of a stent (Wallstent, spiral Z stent) and subsequent brachytherapy (Ir-192, mean dose 30 Gy). 38 patients (group B) had a Wallstent only without subsequent irradiation. Results: The mean survival time of patients (group A) was 291.2 days and 195.1 days (group B). From the statistical evaluation patients who died within one month were eliminated. Conclusion: A combination of percutaneous insertion of a stent and subsequent radiotherapy prolongs significantly the life span of patients with malignant stenosis of the biliary pathways treated by insertion of a metal stent. The most marked difference was recorded in patients with cholangiocarcinoma (339.8 A vs. 298 B).
- MeSH
- Brachytherapy methods MeSH
- Cholangiocarcinoma etiology radiotherapy MeSH
- Humans MeSH
- Pancreatic Neoplasms radiotherapy MeSH
- Gallbladder Neoplasms radiotherapy MeSH
- Prospective Studies MeSH
- Stents methods MeSH
- Bile Ducts pathology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Comparative Study MeSH
- MeSH
- Cholangiocarcinoma etiology MeSH
- Epidemiology MeSH
- Carcinogens MeSH
- Humans MeSH
- Bile Duct Neoplasms epidemiology etiology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
- Geographicals
- Asia MeSH
- Asia, Eastern MeSH
- Europe MeSH
