Materiały źródłowe

• #1 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  Cancer arising from the proximal biliary tree, or hilar cholangiocarcinoma, remains a difficult clinical problem. Complete resection of hilar cholangiocarcinoma is the most effective and only potentially curative therapy, and it now clear that concomitant hepatic resection is required in most cases. Preoperative imaging studies should aim to assess this possibility and must evaluate a number of tumor-related factors that influence resectability. Advances in imaging technology have improved patient selection, but a large proportion of patients are found to have unresectable disease only at the time of exploration. Staging laparoscopy and 13fluoro-deoxyglucose positron emission tomography (FDG-PET) may help to identify some patients with advanced disease; however, local tumor extent, an equally critical determinant of resectability, may be underestimated on preoperative studies. Knowledge of the imaging features of hilar tumors, particularly as they pertain to resectability, is of obvious importance for clinicians managing these patients.

• #2 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/3374/html

  Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. Computed topography (CT), magnetic resonance imaging (MRI), and ultrasound (US) are used to characterize biliary lesions. Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) assess local ductal extent of the tumor while allowing for therapeutic biliary drainage. MRCP has demonstrated similar efficacies to PTC and ERCP in identifying anatomic extension of tumors with less complications. Serum tumor markers, specifically carcinoembryonic antigen (CEA) and CA19-9, are used for the diagnosis, treatment, and monitoring of HC with 89% sensitivity and 86% specificity when combined with other diagnostic modalities. Accurate radiological staging of these lesions is difficult secondary to the complexity of the hilar region, proximity to major vessels, and small tumor sizes. CT accurately predicts HC resectability in 60-90% of cases and is the most frequently used imaging modality to assess biliary tumor resectability. MRI has increasingly gained favor in assessing biliary tumors. The combination of MRI with MRCP is about 80% accurate in predicting HC resectability. Both ERCP and PTC have similar sensitivity (75-85%) and specificity (70-75%) with regard to their ability to attain a tissue diagnosis; however, it is important to note that a negative biopsy cannot be considered definitive and an underlying HC should always be suspected in the right clinical setting regardless of the biopsy results. MRCP has demonstrated similar efficacies to PTC and ERCP in identifying anatomic extension of tumors. The Bismuth-Corlette system provides preoperative assessment of local tumor spread and is used to determine the extent of resection. The American Joint Committee on Cancer (AJCC) is the most commonly used staging system for CC. Margin negative (R0) resection remains the only treatment that offers the chance at long-term survival. Local excision of only the extrahepatic biliary tree should be avoided, as this approach is associated with a high likelihood of an R1 (microscopic) or R2 (macroscopic) resection, as well as worse lymph node clearance and worse survival. In general, 5-year survival after surgical resection of HC ranges from 10% to 40%. Of note, even following an R0 resection, recurrence can be as high as 50-70%. Over the last decade, orthotopic liver transplantation (OLT) has shown promise in the treatment of unresectable HC. The treatment protocol consists of external beam radiation (40-45 Gy), transcatheter radiation (20-30 Gy) via ERCP or PTC with radiosensitizing 5-FU followed by oral capecitabine until the day of transplantation. The current standard chemotherapy regimens for unresectable HC are platinum based in combination with gemcitabine. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. In conclusion, HC is a rare but aggressive disease with a dismal long-term prognosis. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. R0 resection represents the only chance for long-term survival.

• #3 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The diagnosis of hilar cholangiocarcinoma is usually made on evaluation of obstructive jaundice or elevated liver enzymes. Although a small number of patients are diagnosed before the serum bilirubin rises to clinically apparent levels, progressive and unremitting jaundice is the predominant clinical feature in most cases, and diagnostic investigations are largely related to elucidation of the cause of biliary tract obstruction. […] Imaging studies play a critical role in evaluating patients with biliary obstruction and should be directed at fully assessing the extent of disease with a view towards possible resection. In patients with hilar cholangiocarcinoma, evaluation must address four critical components of resectability: (1) level and extent of tumor within the biliary tree; (2) vascular invasion; (3) hepatic lobar atrophy; (4) distant metastatic disease. A combination of studies providing complementary information is typically required to fully assess disease extent.

• #4 Klatskin Tumors (Hilar Cholangiocarcinoma)

  https://my.clevelandclinic.org/health/diseases/hilar-cholangiocarcinoma

  Hilar cholangiocarcinoma is a form of extrahepatic bile duct cancer. In this condition, you have cancerous tumors on part of your bile duct, outside of your liver. These tumors are known as Klatskin tumors. Healthcare providers may be able to cure this condition if they detect and treat tumors before they spread. Surgery, including liver transplant, is the most common treatment. Providers have other treatments that ease symptoms. And researchers are doing several clinical trials to evaluate new treatments. […] If your healthcare provider thinks you may have hilar cholangiocarcinoma, theyll do a physical examination that focuses on your abdomen (belly). Theyll look for signs of fluid buildup, lumps or tenderness in your belly. Theyll examine your skin and the whites of your eyes for jaundice.

• #5 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413

  Endoscopic retrograde cholangiopancreatography (ERCP) uses a dye to highlight the bile ducts on X-ray images. A thin, flexible tube with a camera on the end, called an endoscope, goes through the throat and into the small intestine. The dye enters the ducts through a small hollow tube, called a catheter, passed through the endoscope. Tiny tools passed through the catheter also can be used to remove gallstones. […] If your doctor suspects cholangiocarcinoma, he or she may have you undergo one or more of the following tests: […] Checking the level of carbohydrate antigen (CA) 19-9 in your blood may give your doctor additional clues about your diagnosis. CA 19-9 is a protein that’s overproduced by bile duct cancer cells. […] A high level of CA 19-9 in your blood doesn’t mean you have bile duct cancer, though. This result can also occur in other bile duct diseases, such as bile duct inflammation and obstruction.

• #6 Hilar cholangiocarcinoma – Hancock Health

  https://www.hancockhealth.org/es/mayo-health-library/hilar-cholangiocarcinoma/

  Tumor markers for hilar cholangiocarcinoma include carbohydrate antigen 19-9, also called CA 19-9, and carcinoembryonic antigen, also called CEA. These are proteins that are overproduced by cholangiocarcinoma cells. Highly elevated numbers of these tumor markers may mean a more advanced cancer. […] Having high levels of CA 19-9 and CEA in your blood doesn’t always mean you have hilar cholangiocarcinoma. This also can happen with other bile duct conditions, such as bile duct inflammation and obstruction. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. To get a sample of tissue from a hilar cholangiocarcinoma, a healthcare professional might use a procedure called endoscopic retrograde cholangiopancreatography, also called ERCP. […] If you’re diagnosed with hilar cholangiocarcinoma, the next step is to determine the cancer’s extent, called the stage. Staging tests for hilar cholangiocarcinoma may include additional imaging tests, such as X-ray, MRI, CT and positron emission tomography, which also is called a PET scan.

• #7 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/365065-overview

  Cholangiocarcinomas (CCCs) are malignancies of the biliary duct system that may originate in the liver and extrahepatic bile ducts, which terminate at the ampulla of Vater. CCCs are encountered in three anatomic regions: intrahepatic, extrahepatic (ie, perihilar), and distal extrahepatic. […] Perihilar tumors are the most common CCCs, and intrahepatic tumors are the least common. […] Signs and symptoms of cholangiocarcinoma include the following: Jaundice (most common manifestation), Clay-colored stools, Bilirubinuria (dark urine), Pruritus, Weight loss, Abdominal pain. […] Lab studies: Extrahepatic cholestasis is reflected in elevated levels of conjugated (ie, direct) bilirubin, alkaline phosphatase, and gamma-glutamyltransferase (GGT). Levels of aminotransferases (ie, aspartate aminotransferase [AST], alanine aminotransferase [ALT]) may be normal or minimally elevated.

• #8 Diagnosis of cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2504383/

  Cholangiocarcinoma is suspected based on signs of biliary obstruction, abnormal liver function tests, elevated tumor markers (carbohydrate antigen 19-9 and carcinoembryonic antigen), and ultrasonography showing a bile stricture or a mass, especially in intrahepatic cholangiocarcinoma. […] Magnetic resonance imaging (MRI) or computed tomography (CT) is performed for the diagnosis and staging of cholangiocarcinomas. […] When the diagnosis of a biliary stenosis remains indeterminate at MRI or CT, endoscopic imaging (endoscopic or intraductal ultrasound, cholangioscopy, or optical coherence tomography) and tissue sampling should be carried out. […] The diagnosis of cholangiocarcinoma is particularly challenging in patients with primary sclerosing cholangitis. […] The value of tumor markers in the diagnosis of cholangiocarcinomas remains controversial.

• #9 Diagnosis of cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2504383/

  In unexplained biliary disease, CA 19-9 levels 100 UI/ml are considered suspicious for cholangiocarcinoma in the absence of an inflammatory process. […] The combined use of CEA and CA 19-9 may improve the diagnosis of cholangiocarcinoma, but this has not been reproduced in all studies. […] The sensitivity of fluorodeoxyglucose positron emission tomography is limited in small, infiltrative, and mucinous cholangiocarcinomas. […] The diagnosis of cholangiocarcinoma remains difficult, despite the multiple diagnostic methods available.

• #10 Diagnosis of Cholangiocarcinoma

  https://www.mdpi.com/2075-4418/13/2/233

  The key blood biomarkers for the diagnosis of CCA are carbohydrate antigen 19-9 (CA 19-9) and carcinoembryonic antigen (CEA). […] CA 19-9 is an established blood biomarker for the diagnosis of CCA, with a sensitivity of 50–90 percent and a specificity of 54–98 percent. […] Elevated CA 19-9 at presentation is related to poor prognosis, and considerable rises in CA 19-9 concentrations (>1,000 units/mL) are indicative of unresectable disease. […] In the diagnosis of CCA, a blood CEA level greater than 5.2 ng/mL showed a sensitivity of 68% and a specificity of 82%. […] EUS can assess the regional lymph node status and the local extent of extrahepatic CCA. […] EUS-guided FNA can be performed to acquire tissue samples from tumors and enlarged lymph nodes. […] The combination of an elevated CA 19-9 and a positive brush cytology showed a sensitivity and specificity of 88% and 97%, respectively.

• #11 Tumor markers as a diagnostic key for hilar cholangiocarcinoma | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/2047-783X-15-8-357

  The results of the present study suggest that prognostic information might be obtained from CA19-9 and CEA serum levels before an operative approach for hilar cholangiocarcinoma. Preoperatively obtained CA19-9 serum levels 1000 U/ml and CEA serum levels 14.4 ng/ml are associated with a nonresectability rate of 64% and 53% respectively in comparison to patients with lower preoperative CA19-9 and CEA serum levels respectively. […] This study demonstrates that the amount of serum CA19-9 and CEA is one of the diagnostic tools to estimate resectability rate in correlation to the tumor stage of hilar cholangiocarcinoma.

• #12 Cholangiocarcinoma: Diagnosis, management, and prognosis | Oncohema Key

  https://oncohemakey.com/cholangiocarcinoma-diagnosis-management-and-prognosis/

  Although relatively rare, cholangiocarcinoma is the second most common primary hepatobiliary malignancy in the United States, and its incidence is increasing. […] Locoregional therapies have an important role in the management of cholangiocarcinoma, with radiation therapy more commonly used for hilar cholangiocarcinoma and intraarterial therapy possibly having a role for intrahepatic cholangiocarcinoma. […] The diagnosis of cholangiocarcinoma relies on a combination of laboratory, cytology, and imaging studies. […] Diagnosis of hilar cholangiocarcinoma relies on multiple methods of assessment. Ultrasonography is often the first mode of imaging used to evaluate a patient with jaundice, but is of little help except in identifying biliary dilatation. […] Most clinicians believe that MRI and magnetic resonance cholangiopancreatography (MRCP) are still superior to CT in the diagnosis and determination of resectability of hilar cholangiocarcinoma.

• #13 Radiological diagnosis and staging of hilar cholangiocarcinoma

  https://www.wjgnet.com/1948-5204/full/v5/i7/115.htm

  Hilar cholangiocarcinoma is a rare malignant tumor arising from the epithelium of the bile ducts. Surgery is still the only chance of potentially curative treatment in patients with perihilar cholangiocarcinoma. […] Accurate diagnosis and staging of these tumors is therefore critical for optimal treatment planning and for determining a prognosis. Multidetector computed tomography (MDCT), magnetic resonance imaging (MRI) and MR cholangiography are useful tools, both to diagnose and stage hilar cholangiocarcinoma. […] The role of cholangiography in the evaluation of hilar cholangiocarcinoma is two-fold: to assess tumoral extension to identify potentially resectable patients and to help in planning palliative biliary drainage in non-resectable patients. […] MDCT allows for faster scanning with thinner collimation and results in an improved diagnosis and staging hilar cholangiocarcinoma.

• #14 Radiological diagnosis and staging of hilar cholangiocarcinoma

  https://www.wjgnet.com/1948-5204/full/v5/i7/115.htm

  Diagnostic features of HCCA include intrahepatic segmental biliary dilatation, periductal thickening, endoluminal lesions and direct tumor spread to the liver or adjacent vessels. […] The cornerstone of oncological resection in the liver is to resect the whole tumor with free margins but still leave enough liver to maintain hepatic function. […] Preoperative staging should focus on biliary, vascular, hepatic, lymph node and extrahepatic extension. […] Different imaging techniques are currently used for the preoperative staging of hilar cholangiocarcinoma. However, accurate staging remains a challenge.

• #15 British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma | Gut

  https://gut.bmj.com/content/73/1/16

  Consideration should be given to possible benign causes of biliary tract stricturing/obstruction during MDT discussion, in correlation with appropriate serological investigations and clinical history, to ensure that alternative diagnoses are considered while a pathological diagnosis of CCA is secured. […] Contrast enhanced multiphasic CT of the chest, abdomen and pelvis to stage the primary tumour, including assessment of local vascular relationships, should be undertaken for all types of CCA. […] Contrast enhanced MRI and magnetic resonance cholangiopancreatography (MRCP) should be undertaken for perihilar and intrahepatic tumours to better delineate the extent of biliary involvement and identify any satellites/intrahepatic metastases. […] For tumours involving the more distal extrahepatic duct, MRI is unlikely to add any further information over and above CT.

• #16 British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma | Gut

  https://gut.bmj.com/content/73/1/16

  18F-fluoro-deoxy-glucose positron emission tomography (18FDG-PET) CT for detection of nodal and distant metastatic disease is recommended as part of staging investigations. […] Ultrasound (US) or CT-guided biopsy of the primary intrahepatic tumour or metastatic lesions should be undertaken to acquire a pathological diagnosis following MDT discussion and consensus. […] Before undertaking any endoscopic investigations for a suspected CCA, all patients should have undergone a triple-phase CT scan of the abdomen/pelvis and chest along with dynamic MRI and MRCP if proximal biliary obstruction is suspected. […] Patients with operable distal malignant tract obstruction (DMTO) should undergo a combination of endoscopic US and endoscopic retrograde cholangiopancreatography (ERCP) to try to confirm a malignant histological diagnosis before proceeding to surgery.

• #17 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413

  During endoscopic retrograde cholangiopancreatography (ERCP), a thin, flexible tube equipped with a tiny camera is passed down your throat and through your digestive tract to your small intestine. The camera is used to examine the area where your bile ducts connect to your small intestine. Your doctor may also use this procedure to inject dye into the bile ducts to help them show up better on imaging tests. […] Imaging tests can help your doctor see your internal organs and look for signs of cholangiocarcinoma. Techniques used to diagnose bile duct cancer include ultrasound, computerized tomography (CT) scans and magnetic resonance imaging (MRI) combined with magnetic resonance cholangiopancreatography (MRCP). MRCP is increasingly being used as a noninvasive alternative to ERCP. It offers 3D images without the need for a dye to enhance the images.

• #18 National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

  https://www.wjgnet.com/1007-9327/full/v30/i9/1018.htm

  The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions. […] Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA. […] The basis of guidelines is the literature review of randomised controlled trials (RCTs), meta-analyses, case cohorts and prospective and retrospective studies. […] The necessity of establishing a tissue diagnosis before surgery depends upon the clinical situation. It is not critical for planning surgery in patients with characteristic findings of mass-forming malignant biliary obstruction and may not be necessary for planning palliative therapy. […] Given the complexity of diagnosis and staging, each case of suspected hCCA should be discussed in a multidisciplinary team (MDT) meeting. […] ERCP guided brush cytology and targeted biopsy should be the preferred diagnostic modality to obtain histological confirmation in suspicious or indeterminate biliary strictures. […] The initial radiological examination should be a cross-sectional imaging study, such as a CT or MRI and MRCP.

• #19 Endoscopic Ultrasound in the Diagnosis of Extrahepatic Cholangiocarcinoma: What Do We Know in 2023?

  https://www.mdpi.com/2075-4418/13/6/1023

  The purpose of this article is to summarize the current data regarding the role of endoscopic ultrasound (EUS) and EUS-tissue acquisition in the diagnostic algorithm of bile duct tumors. […] EUS may be helpful in the setting of bile duct dilation if no mass is seen on CT or MRI, and unnecessary ERCP can be avoided in about one-third of the patients. EUS evaluation of the biliary tree is performed from the level of the duodenal bulb and the distal part of the gastric antrum, for both biliary strictures and CCAs. Using EUS, they can be visualized either as a mass or as a biliary stricture. […] The EUS aspect suggestive of CCA is a mass extending beyond the bile duct wall or periductal infiltration, with a wall thickness of more than 3 mm, or an intraductal mass-growing lesion. In previous research, distal tumors which were closer to the EUS transducer were diagnosed in 100% of the cases, while tumors located further from the transducers were only diagnosed in 83% of the cases. Overall, EUS performed better in identifying tumors in comparison to CT or MRI. Extrahepatic CCAs were diagnosed at an early stage when MRCP was followed by EUS (sensitivity 90% and specificity 98%). EUS is also useful when assessing common bile duct dilatation associated with normal hepatic tests and inconclusive imaging.

• #20 Endoscopic Ultrasound in the Diagnosis of Extrahepatic Cholangiocarcinoma: What Do We Know in 2023?

  https://www.mdpi.com/2075-4418/13/6/1023

  EUS proved a T staging accuracy of 60–81%, while intraductal ultrasound (IDUS) can assess the T staging with 68% accuracy. […] The N staging accuracy varies between 66 and 81%. EUS can identify nodal involvement with a higher accuracy than cross-sectional imaging. The presence of malignant regional lymph nodes precludes curative oncological resection or liver transplant for CCA, and is associated with a four-fold higher risk of death. […] EUS can identify and provide additional information about biliary strictures, without etiology confirmed by other imaging modalities. EUS can specify their location (distal/medium/proximal common bile duct) and their malignant characteristics, and may detect and stage tumors. […] EUS-guided tissue sampling showed better diagnostic accuracy in comparison with ERCP. When both were used, the sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, and accuracy were 86, 98, 12.50, 0.17, and 96.5%, respectively.

• #21 Bile Duct Cancer Diagnostic Tests | How Do You Test for Bile Duct Cancer? | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/detection-diagnosis-staging/how-diagnosed.html

  A cholangiogram is an imaging test that looks at the bile ducts to see if they’re blocked, narrowed, or dilated (widened). This can help show if someone might have a tumor that’s blocking a duct. […] Imaging tests might suggest that a bile duct cancer is present, but in many cases, samples of bile duct cells or tissue are removed (biopsied) and looked at with a microscope to be sure of the diagnosis. […] There are many ways to take biopsy samples to diagnose bile duct cancer. […] For this test, a thin, hollow needle is put through the skin and into the tumor without making a cut in the skin. […] Along with looking at the biopsy samples with a microscope to see if they contain cancer cells, other lab tests might also be done on the samples.

• #22 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413

  A biopsy is a procedure to remove a small sample of tissue for examination under a microscope. […] If the suspicious area is located very near where the bile duct joins the small intestine, your doctor may obtain a biopsy sample during ERCP. If the suspicious area is within or near the liver, your doctor may obtain a tissue sample by inserting a long needle through your skin to the affected area (fine-needle aspiration). […] If your doctor confirms a diagnosis of cholangiocarcinoma, he or she tries to determine the extent (stage) of the cancer. Often this involves additional imaging tests. Your cancer’s stage helps determine your prognosis and your treatment options. […] Don’t hesitate to ask about your doctor’s experience with diagnosing cholangiocarcinoma. If you have any doubts, get a second opinion.

• #23 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  Currently, there is no clinical staging system available that stratifies patients preoperatively into subgroups based on potential for resection. The modified BismuthCorlette classification stratifies patients based only on the extent of biliary involvement by tumor, and the AJCC staging system is based largely on pathological criteria and has little applicability for preoperative staging. […] Long-term survival with hilar cholangiocarcinoma depends critically on a complete resection with histologically negative resection margins. Proper patient selection requires high quality, complementary imaging studies and careful consideration of all of the available data.

• #24 Hilar cholangiocarcinoma (bile duct cancer): stage, treatment, surgery | Dr. Nikhil Agrawal

  https://www.drnikhilagrawal.com/bile-duct-cancer-cholangiocarcinoma-hilar

  Computed tomographic (CT) scan and magnetic resonance imaging (MRI): In CT the patient is scanned with a beam of x-rays, which is then computer-processed. MRI uses strong magnetic fields, radio waves and computer to generate the view of the inside of your body. […] Cholangiography: Endoscopic retrograde cholangiography (ERC) or percutaneous transhepatic cholangiography (PTC) provides excellent visualization of the biliary tree by injecting contrast into the biliary tree and taking images with the help of X-ray beams. […] Biopsy or cytology: It is a procedure in which small bits of tissue or cells are obtained from the tumour by passing a needle under radiologic guidance. […] Staging tells us the current extent of the disease. The staging informs clinicians about the size of the tumour, its exact location, how much they have spread locally and whether they have spread to distant organs.

• #25 Hilar cholangiocarcinoma (bile duct cancer): stage, treatment, surgery | Dr. Nikhil Agrawal

  https://www.drnikhilagrawal.com/bile-duct-cancer-cholangiocarcinoma-hilar

  The staging of hilar cholangiocarcinoma deepens on the extent of the tumour, extension into the bile duct on the right side and the left side and the relation of the tumour to the blood supply of liver (hepatic artery and portal vein and their right and left branches). […] TNM (Tumour, Node and Metastasis) classification developed by the American Joint Committee on Cancer (AJCC) is used for accurate classification of stage and it spans from I to IV. […] The treatment of hilar cholangiocarcinoma depends on the stage at which it is detected. Surgical excision, when possible, is the best therapeutic option for hilar cholangiocarcinoma as it offers the possibility of a cure. […] In cases of locally advanced hilar cholangiocarcinoma that cannot be surgically removed and have not spread to other parts of the body, a liver transplant can be considered as a treatment option.

• #26 Clinical manifestations and diagnosis of cholangiocarcinoma – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-cholangiocarcinoma

  The most recent (2017) revision of the tumor, node, metastasis (TNM) classification no longer considers type IV tumors to represent a T4 primary tumor. […] Hilar cholangiocarcinoma as seen on MRCP. […] Cholangiocarcinoma of the common bile duct, as seen during an ERCP procedure. […] Stricture at the hepatic duct bifurcation. […] Distal common bile duct stricture.

• #27 British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma | Gut

  https://gut.bmj.com/content/73/1/16

  These guidelines for the diagnosis and management of cholangiocarcinoma (CCA) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included a multidisciplinary team of experts from various specialties involved in the management of CCA, as well as patient/public representatives from AMMF (the Cholangiocarcinoma Charity) and PSC Support. Quality of evidence is presented using the Appraisal of Guidelines for Research and Evaluation (AGREE II) format. The recommendations arising are to be used as guidance rather than as a strict protocol-based reference, as the management of patients with CCA is often complex and always requires individual patient-centred considerations. […] The management of CCA should be undertaken at centres with expertise across all relevant specialties, including surgery, interventional radiology, endoscopy, hepatobiliary medicine, oncology and pathology.

• #28 British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma | Gut

  https://gut.bmj.com/content/73/1/16

  All patients with CCA discussed at multidisciplinary team (MDT) meetings should be classified as best as possible into either intrahepatic, perihilar or distal CCA. This should be clearly recorded in the MDT outcome discussion. […] The requirement to have tissue available for molecular profiling to inform treatment decisions should be considered when immunohistochemistry is planned on lesional biopsy material. […] A diagnosis of combined hepatocellular-CCA should be made on morphological pathological grounds only. […] All centres managing patients with CCA should have clear established diagnostic pathways for patients presenting with jaundice/biliary obstruction, with streamlined transition to local and regional hepato-pancreato-biliary (HPB) MDT meetings. […] Having completed imaging, all patients should undergo a detailed review of clinical presentation, examination findings, blood investigations and imaging, ideally at a regionally coordinated hepatobiliary MDT meeting, with prompt assessment of the results and communication with the patient.

• #29 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes. CCAs are usually asymptomatic in early stages and, therefore, often diagnosed when the disease is already in advanced stages, which highly compromises therapeutic options, resulting in a dismal prognosis. As no specific CCA radiology pattern exists, histopathological or cytological analysis is mandatory to confirm the diagnosis. This diagnosis is based on the WHO classification of biliary tract cancer showing an adenocarcinoma or mucinous carcinoma, with tubular and/or papillary structures and a variable fibrous stroma. The most frequent imaging patterns displayed by iCCA on both CT and MRI are arterial peripheral rim enhancement with progressive homogeneous contrast agent uptake until the delayed or stable uptake during late dynamic phases. Imaging techniques, such as ultrasonography, contrast-enhanced ultrasonography (CEUS), CT and MRI, play a key part in the management of CCA in terms of diagnosis, staging, follow-up and assessment of treatment response. Histologically, although the vast majority of pCCA and dCCA are conventional mucin-producing adenocarcinomas or papillary tumours, iCCA shows several histological variants. The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. The age-standardized incidence of CCA shows considerable geographical variation, with the highest incidence in Eastern countries/regions; incidence varies from 85 per 100,000 in northeastern Thailand (the highest reported value globally) to 0.4 per 100,000 in Canada. Furthermore, there is substantial global variation in rates of microscopically verified cases of CCA reported to cancer registries worldwide. CCA can be notoriously difficult to accurately diagnose due to its location often being inaccessible to histology or cytology, a lack of clear diagnostic imaging criteria, and inaccurate non-invasive tumour biomarkers.

• #30 Pathology Pearls: Intrahepatic Cholangiocarcinoma | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/pathology-pearls/pathology-pearls-intrahepatic-cholangiocarcinoma

  A targeted biopsy of the mass is performed. […] In the clinical context of a single central intrahepatic lesion with absence of lesions in the rest of the body, the histologic findings are compatible with intrahepatic cholangiocarcinoma. […] Intrahepatic cholangiocarcinoma is the second most common primary hepatic malignancy after hepatocellular carcinoma. […] ICCAs are exclusively adenocarcinomas with ductal or tubular pattern showing variably sized lumen. […] Grading of intrahepatic cholangiocarcinomas is based on morphology. […] The main differential diagnoses include metastatic tumors from other parts of the gastrointestinal tract or the rest of the body as well as hepatocellular carcinomas (HCCs). […] A panel of immunohistochemical stains should be used to differentiate intrahepatic cholangiocarcinomas from other differentials as follows: […] CK7 (90%) and CK19 (80 to 90%) are positive in most cholangiocarcinomas and CK20 can be positive in peripherally located cholangiocarcinomas. […] TTF-1 and Napsin can be positive in hilar cholangiocarcinomas.

• #31 Multimodal molecular imaging evaluation for early diagnosis and prognosis of cholangiocarcinoma | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-01147-7

  Molecular imaging has higher sensitivity and specificity than conventional imaging for the diagnosis of cholangiocarcinoma. […] Different molecular imaging methods have specific advantages for cholangiocarcinoma diagnosis. […] Molecular imaging has great potential for finding targets, probe synthesis, and clinical applications in cholangiocarcinoma. […] CCA is diagnosed via a combination of clinical symptoms, imaging manifestations, biochemical features, and histological examinations, and imaging plays a crucial role. […] CT is considered the standard imaging modality for detecting CCA features and for staging. […] MRI is superior to CT for diagnosis and staging, and is comprises specific sequences such as diffusion-weighted imaging and MRCP. […] PET scan imaging is used to assess and evaluate distant metastasis.

• #32 Multimodal molecular imaging evaluation for early diagnosis and prognosis of cholangiocarcinoma | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-01147-7

  Molecular imaging is an emerging discipline at the intersection of molecular biology and traditional medical imaging. […] Compared with traditional imaging techniques, molecular imaging can detect the specific histopathological changes at the cellular and molecular levels before the morphological changes of the disease take place. […] This technology has the advantage of finding smaller lesions while simultaneously providing a basis for differential diagnosis and curative effect evaluation. […] This allows a more accurate diagnosis of the disease. […] In the near future, it is expected that molecular imaging techniques will be used to study the pathogenesis of CCA in more detail to identify the key components of the onset stage, thereby providing an early and definitive diagnosis of CCA.

• #33 Comprehensive management of cholangiocarcinoma: Part I. Diagnosis

  https://www.ijgii.org/journal/view.html?doi=10.18528/gii1500341

  FISH is a cytogenetic technique that uses fluorescent DNA probes to assess for specific DNA sequences under a fluorescent microscope. […] Per-oral cholangioscopy (POCS) enables biopsies of the target tissue under direct visualization and aims to facilitate the diagnosis of biliary strictures. […] Intraductal US (IDUS) is used in order to increase the sensitivity of intraductal biopsies during ERCP. […] Probe-based confocal laser endomicroscopy (pCLE) is a new technique that provides in vivo real time images of the tissue under examination. […] Serum and bile tumor markers are not specific for any type of CC.

• #34 Cholangiocarcinoma: Symptoms, Causes, Types, Staging & Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact

  https://www.pacehospital.com/cholangiocarcinoma-bile-duct-cancer-symptoms-causes-types-staging-treatment

  Since cholangiocarcinoma often presents with non-specific symptoms, early and accurate diagnosis requires a combination of imaging, laboratory tests, and histopathological confirmation. […] Elevated bilirubin, alkaline phosphatase (ALP), and gamma-glutamyl transferase (GGT) suggest bile duct obstruction. […] CA 19-9 (Carbohydrate Antigen 19-9): Elevated in 50–75% of patients with CCA but also increased in benign biliary diseases. […] MRI/MRCP (Magnetic Resonance Cholangiopancreatography): The best imaging modality for visualizing bile ducts and identifying strictures or masses. […] ERCP (Endoscopic Retrograde Cholangiopancreatography): Used for bile duct visualization, biopsy, and stent placement in cases of obstructive jaundice. […] Molecular and Genetic Testing: IDH1/IDH2, FGFR2, and BRAF mutations: Guide targeted therapies in advanced cases.

• #35 Multimodal molecular imaging evaluation for early diagnosis and prognosis of cholangiocarcinoma | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-01147-7

  This article reviews clinical and preclinical studies on the application of different molecular imaging modalities in CCA over the past two decades. […] The aim of this study was to describe and discuss the role and characteristics of different molecular imaging methods for CCA diagnosis and research. […] It is expected to improve the accuracy of early diagnosis of CCA, establish the best treatment strategies, and ameliorate the quality of life and prognosis of patients.

Zobacz więcej