Extrahepatic cholangiocarcinoma is a rare disease with poor prognosis, requiring comprehensive multidisciplinary management. Only radical resection gives hope for long-term survival. Most patients have either an unresectable disease or their condition does not allow for radical surgery. The method of choice for resectable perihilar cholangiocarcinoma is en-block liver resection including the extrahepatic bile duct, or pancreatoduodenectomy for distal cholangiocarcinoma. Hepatopancreatoduodenectomy can be performed in selected patients if the entire hepato-choledochus is affected. Liver transplantation after neoadjuvant treatment can be also considered in highly selected patients with unresectable perihilar cholangiocarcinoma. These procedures are technically demanding, are associated with high morbidity and relevant mortality, and must be concentrated in tertiary hepatobiliary centers. Preoperative optimization (bile duct drainage, nutrition, portal venous embolization, etc.) plays a crucial role in reducing postoperative morbidity and mortality.

• Klíčová slova
• Hepatectomy, bile duct, cholangiocarcinoma, extrahepatic, pancreatoduodenectomy,
• MeSH
• cholangiokarcinom * patologie   chirurgie   MeSH
• hepatektomie metody   MeSH
• Klatskinův nádor * chirurgie   MeSH
• lidé MeSH
• nádory žlučových cest * chirurgie   patologie   MeSH
• žlučové cesty extrahepatické * chirurgie   patologie   MeSH
• žlučové cesty intrahepatální patologie   chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Cholangiocellular carcinoma (CCC) is a malignant tumor harboring a poor prognosis, occurring in the liver, gallbladder and in extra- or intrahepatic biliary ducts. The article reviews the topic concerning CCC from the point of view of a surgical pathologist. The paper deals with classification of CCC into an intrahepatic/peripheral and hilar/extrahepatic subtype with different morphology, molecular features and prognosis; together with classical gross pathology, histopathology and natural history of CCC. Hilar and extrahepatic CCC share some biological characteristics with pancreatic ductal adenocarcinoma. The review comprises various types of precancerous lesions of biliary tract, summarizes updates in 8th edition of TNM classification and describes the routine issues concerning histopathological diagnostics of CCC, including immunohistochemistry and frozen section methods.

• Klíčová slova
• cholangiocellular carcinoma, extrahepatic, hilar, intrahepatic, pathology,
• MeSH
• cholangiokarcinom * patologie   MeSH
• duktální karcinom slinivky břišní * patologie   MeSH
• lidé MeSH
• nádory žlučových cest * patologie   MeSH
• prognóza MeSH
• žlučové cesty intrahepatální * patologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

Hilar cholangiocarcinoma is a rare tumor with a rather poor prognosis, and thus remains a challenge for diagnosis and treatment. The sole potentionally curative treatment is the complete resection of the tumor. A negative surgical margin is one of the most important factors in achieving prolonged survival. A preoperative evaluation of the tumor is important for the evaluation of resectability and the extent of surgery. Unfortunately, only a small number of patients with hilar cholangiocarcinoma is indicated for the radical procedure. Liver transplantation is not a standard method in the treatment of the Klatskin tumor and it is reserved only for carefully selected patients in a few transplant centres. The main aim of the palliative treatment is biliary drainage, reduction of the pain and pruritus and overall improvement of the quality of life. Adjuvant chemotherapy and radiotherapy are the important parts of the complex therapy, however, no definite regimen has been established to date. Further evidence is needed to define the role of liver transplantation and (neo)adjuvant new therapeutic modalities. Key words: hilar cholangiocarcinoma - radical surgery - palliative treatment - liver transplantation - (neo) adjuvant chemotherapy - radiotherapy.

• MeSH
• ductus hepaticus communis * MeSH
• Klatskinův nádor diagnóza   terapie   MeSH
• lidé MeSH
• nádory žlučových cest diagnóza   terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND: Hilar cholangiocarcinoma (HC), also referred to as Altemeier-Klatskins tumour, is a lethal primary extrahepatic carcinoma of biliary epithelial origin, arising within 2cm of the hilar confluence. Radical surgical excision provides the best chance for a cure; however, the management of patients with HC is challenging not only because of the need for a high level of skill in biliary and hepatic resections, but also because of the difficulty in reaching an accurate diagnosis preoperatively. In fact, the differential diagnosis of HC is a diagnostic dilemma which is currently persisting, as modern, sophisticated diagnostic modalities are not always able to provide a definitive preoperative diagnosis. This difficulty is compounded by the fact that alternative entities that mimic HC may be present in up to 25% of patients with hilar obstruction. This makes precise preoperative characterisation of a hilar stricture extremely important by preventing unnecessary, high-risk, major surgical procedures. Therefore, alternative benign entities masquerading as Altemeier-Klatskins tumour deserve an important place in the differential diagnosis of hilar obstruction. PURPOSE: Considering the important clinical implications that a precise diagnosis of the aetiological cause of a biliary obstruction at the liver hilum would have, this paper will focus on the differentiation between HC and benign hilar obstructions and will review benign tumours and pseudotumours masquerading as HC along with their specific diagnostic features.

• Klíčová slova
• Altemeier-Klatskin’s tumour, Klatskin-mimicking lesions, benign Altemeier-Klatskin-like lesions, hilar biliary stricture, misdiagnosis,
• MeSH
• diferenciální diagnóza MeSH
• játra patologie   MeSH
• Klatskinův nádor diagnóza   MeSH
• lidé MeSH
• nádory žlučových cest diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

Cholangiocarcinoma (CCA) is a liver malignancy associated with a poor prognosis. Its main subtypes are peripheral/intrahepatic and hilar/extrahepatic CCA. Several molecular, morphological and clinical similarities between hilar/extrahepatic CCA and pancreatic ductal adenocarcinoma (PDAC) have been described. FOXF1 is a transcription factor which has been described to have prognostic significance in various tumors and it is involved in the development of bile ducts. The aim of this study is to determine occurrence of nuclear expression of FOXF1 in both subtypes of CCA and metastatic PDAC and assess its potential usefulness as a diagnostic marker. Secondary aims were to investigate the use of C-reactive protein (CRP) immunohistochemistry for diagnosing intrahepatic peripheral CCA and the significance of histological features in CCA subtypes. 32 archive specimens of CCA, combined hepatocellular carcinoma-CCA (HCC-CCA) and liver metastasis of PDAC were stained by FOXF1 and CRP immunohistochemistry and evaluated to determine histological pattern. The CCAs were classified radiologically into peripheral/intrahepatic and hilar subtype. Using Fisher exact test, we identified nuclear FOXF1 as a fairly specific (87%) but insensitive (65%) marker of hilar and extrahepatic CCA and metastatic PDAC (p = 0.005). CRP immunohistochemistry was characterized by a high sensitivity and specificity, of 79% and 88%, respectively (p = 0.001). We did not identify any histomorphological features associated with either types of CCA or metastatic PDAC. As a conclusion of novel finding, FOXF1 immunohistochemistry may be regarded as a specific but insensitive marker of hilar/extrahepatic CCA and metastatic PDAC and it may help distinguish them from peripheral CCA.

• Klíčová slova
• Foxf1, cholangiocarcinoma, extrahepatic, hilar, intrahepatic cholangiocarcinoma, peripheral,
• MeSH
• dospělí MeSH
• duktální karcinom slinivky břišní metabolismus   sekundární   MeSH
• forkhead transkripční faktory metabolismus   MeSH
• hepatocelulární karcinom metabolismus   patologie   MeSH
• imunohistochemie MeSH
• Klatskinův nádor metabolismus   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití MeSH
• nádorové biomarkery metabolismus   MeSH
• nádory jater metabolismus   sekundární   MeSH
• nádory slinivky břišní metabolismus   patologie   MeSH
• nádory žlučových cest metabolismus   patologie   MeSH
• následné studie MeSH
• prognóza 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
• Názvy látek
• forkhead transkripční faktory MeSH
• FOXF1 protein, human MeSH Prohlížeč
• nádorové biomarkery 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

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

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

Bile duct malignancies include intrahepatic cholangiocarcinoma (ICC), extrahepatic cholangiocarcinoma (ECC), gall bladder carcinoma (GC) and carcinoma of Vater's ampulla (ampulloma). Bile duct neoplasms are rare tumours with overall poor prognosis. The overall incidence affects up to 12.5 per 100,000 persons in the Czech Republic. The mortality rate has risen recently to 9.5 per 100,000 persons. The incidence and mortality have been remarkably stable over the past 3 decades. The survival rate of patients with these tumours is poor, usually not exceeding 12 months. The diagnostic process is complex, uneasy and usually late. Most cases are diagnosed when unresectable, and palliative treatment is the main approach of medical care for these tumours. The treatment remains very challenging. New approaches have not brought much improvement in this field. Standards of palliative care are lacking and quality of life assessments are surprisingly not common. From the scarce data it seems, however, that multimodal individually tailored treatment can prolong patients'survival and improve the health-related quality of life. The care in specialized centres offers methods of surgery, interventional radiology, clinical oncology and high quality supportive care. These methods are discussed in the article in greater detail. Improvements in this field can be sought in new diagnostic methods and new procedures in surgery and interventional radiology. Understanding the tumour biology on the molecular level could shift the strategy to a more successful one, resulting in more cured patients. Further improvements in palliative care can be sought by defining new targets and new drug development. The lack of patients with bile duct neoplasms has been the limiting factor for any improvements. A new design of larger randomized international multicentric clinical trials with prompt data sharing could help to overcome this major problem. Defining standards of palliative care is a necessity. Addressing health-related quality of life could help to assess the real benefit of palliative treatment.

• MeSH
• cholangiokarcinom diagnóza   epidemiologie   terapie   MeSH
• lidé MeSH
• nádory ductus choledochus diagnóza   terapie   MeSH
• nádory žlučníku diagnóza   terapie   MeSH
• nádory žlučových cest * diagnóza   epidemiologie   terapie   MeSH
• prognóza MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH

INTRODUCTION: The prognosis of extrahepatic cholangiocarcinoma is dismal and the only way to achieve long-term survival is surgical resection. While pancreatoduodenectomy (PD) is the standard procedure for distal cholangiocarcinoma (distal bile duct cancer; DBDC), bile duct segmental resection (BDR) can be used as an alternative approach for middle bile duct cholangiocarcinoma (middle bile duct cancer; MBDC). The aim of the study was to calculate the short-term and long-term outcomes of curative-intent surgery in distal bile duct cholangiocarcinoma patients. METHODS: A retrospective cohort study of consecutive patients treated for MBDC and DBDC with PD or BDR between 1/2009-12/2019. The patients were divided according to the type of surgical resection (PD and BDR group). Demographic, clinicopathological and histopathological data and overall survival (OS) were evaluated in both groups. OS was estimated using the Kaplan-Meier analysis. RESULTS: The study comprised a total of 62 patients - 45 patients (72.6%) in the PD group and 17 (27.4%) in the BDR group. Patients undergoing BDR were significantly older than those receiving PD (p=0.048). Men predominated in the PD group (N=34/45; 75.6%) while more women were included in the BDR group (N=10/17; 58.8%). Median age was higher in the BDR group (p=0.048). Serious morbidity (Clavien-Dindo III-V) (33.3% vs 11.8%), 30-day and 90-day mortality (4.4% vs 0.0% and 8.9% vs 5.9%, respectively) predominated in the PD group although the differences were not statistically significant, as well as a longer hospital stay (16.0 days vs 11.0 days; p=0.002). Pathological assessments revealed comparable numbers of positive lymph nodes in both groups, but a significantly higher number of total resected lymph nodes in the PD group (p.

• Klíčová slova
• bile duct resection, distal bile duct cancer, middle bile duct cancer, pancreatoduodenectomy,
• MeSH
• cholangiokarcinom * chirurgie   patologie   MeSH
• lidé MeSH
• nádory žlučových cest * chirurgie   patologie   MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• žlučové cesty extrahepatické * patologie   chirurgie   MeSH
• žlučové cesty intrahepatální patologie   chirurgie   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH