All types of cancerous growth in the biliary duct are termed as cholangiocarcinoma. This finding may be due in part to the narrow pathologic margins. Klatskin tumor is quite rare, more in male sex in age 60 years. Sonographic features of these tumors include a dilatation of the intrahepatic biliary ducts but not the extrahepatic duct, b nonunion of the right and left hepatic ducts, and c small, solid masses at. Feb 08, 2018 the objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with hilar cholangiocarcinoma. Klatskin tumors are are a type of cholangiocarcinoma that begins in an area called the hilum, where the left and right bile ducts join and leave the liver. It has been suggested that preoperative endoscopic retrograde cholangiopancreatography ercp with biliary drainage in patients with tumors of the bile duct increases the risk of implantation metastases. Vincenzkrankenhaus in datteln, presenting with a prediagnosed klatskintumor bismuth iv with worsening general condition. Ebd was the first biliary decompression procedure performed in 87 patients. How to help a friend with a klatskin tumor diagnosis by dr. A klatskin tumor is a tumor which forms in the upper region of the bile duct, where the left and right bile ducts meet.
A, longitudinal view of the common hepatic duct shows a tapered segment arrow consistent with tumor stricture. Klatskin tumors, or hilar cholangiocarcinomas, are rare in the united states population but they may have a rising incidence. Endoscopic or percutaneous biliary drainage for klatskin. Endoscopic retrograde cholangiopancreatography and endoscopic endoprosthesis insertion have a limited value in the management of patients with klatskin tumors. Optimal biliary drainage for inoperable klatskins tumor based on bismuth type world j gastroenterol, 2007, pp. Treatment and longterm prognosis are dependent upon the location. How to help a friend with a klatskin tumor diagnosis. Healthy patients with small, minimally involved tumors may. This presentation will focus on new approaches for surgical treatment of klatskin tumours and its impact on the criteria used for resectability of this dif. Hepatic resection and transplantation for peripheral cholangiocarcinoma. Demographics, tumor characteristics, treatment, and. Research helps us better understand diseases and can lead to advances in diagnosis and treatment.
Surgical resection with a tumorfree margin is the only curative treatment for hilar cholangiocarcinoma klatskin tumor. There is prooved connection between this malignant tumor and primary sclerosis cholangitis autoimmune disease and parasitic infection in biliary ducts clonorchis sinensis and opisthorchis viverrini, both in southeast asia. Klatskin tumor is a term that was traditionally given to a hilar cholangiocarcinoma, occurring at the bifurcation of the common hepatic duct. Mark fraiman on april 20, 2016 in bile cancer, bile duct cancer if someone close to you, say a family member or friend, has recently been diagnosed with bile duct cancer, specifically involving klatskin tumors, you may be struggling with what to say or how best to help. The size of the tumor at the time of diagnosis affects a patients prognosis of a klatskin tumor. A klatskin tumor or hilar cholangiocarcinoma is a cholangiocarcinoma cancer of the biliary tree occurring at the confluence of the right and left hepatic bile ducts. Symptoms usually dont present until advanced stages of disease, when jaundice is the most common feature. Treatment consists of surgery, radiation, chemotherapy and. Abstract background the objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with hilar cholangiocarcinoma. A case of inoperable klatskin tumor showing response to wild. This section provides resources to help you learn about medical research and ways. I am 61 years old, married and we have two wonderful grownup sons.
An electronic database search, including pubmedmedline, was performed using the terms hilar cholangiocarcinoma and klatskins tumor. Jul 23, 20 klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. Mar 27, 2005 hi jackie, after several echographic tests and ctscans there was still nothing visualized. This neoplasia, which originates in the epithelium of the biliary ducts, or cholangiocarcinoma cc, represents 10% of hepatobiliary tumours and 2% of malignant tumours.
Listing a study does not mean it has been evaluated by the u. Because the drugs reach all the areas of the body, this is known. Endoscopic retrograde cholangiopancreatography and endoscopic. Endoscopic retrograde cholangiopancreatography ercp is frequently used in the management of patients with malignant hilar biliary obstruction that includes klatskin tumor. A, klatskins tumor tumor located in the hepatic duct bifurcation in a patient with primary. Visit the orphanet disease page for more information. Klatskin tumor genetic and rare diseases information. Apr 20, 2020 the use of hormones in treatment, including somatostatin analogues, cholecystokinin, and cholecystokinin antagonists, is being investigated. Klatskin tumors, or hilar ccas, are bile duct tumors that involve the common hepatic duct bifurcation but may arise from the intrahepatic ihcca. Klatskin tumour is a type of cholangiocarcinoma or a tumour of the bile duct system.
He said, hi, i have a 63yearold patient here with painless obstructive jaundice. We further evaluate differences between extra and intrahepatic klatskin tumors, as prior reports suggest that they tend to present and behave differently. Treatment ofhilar cholangiocarcinoma klatskin tumors with hepatic resection or transplantation shunzaburo iwatsuki, md, rho. The incidence of klatskin tumors increases with age and patients younger than 20 years of age are rare. These drugs enter the bloodstream and reach all areas of the body, making this treatment useful for some cancers that have spread to organs beyond the bile duct. We offer a widget that you can add to your website to let users look up cancerrelated terms. Oct, 2016 klatskin tumors are are a type of cholangiocarcinoma that begins in an area called the hilum, where the left and right bile ducts join and leave the liver. Hilar cholangiocarcinoma, endoscopic retrograde cholangiography, nuclear magnetic resonance cholangiog raphy, hepatobiliary surgery. Endoscopic retrograde cholangiopancreatography and. Tests that examine the bile ducts and nearby organs are used to detect find, diagnose, and stage. The patients receiving metallic stents had better survival than those receiving plastic stents 40. Guidelines for the diagnosis and management of intrahepatic. Dec 11, 2015 a closer look at klatskin tumor treatment options by dr.
A new clinical classification of hilar cholangiocarcinoma. Of those cases, roughly twothirds are located somewhere in the bile duct system, with an additional 10% containing klatskin tumors, a serious and rare type of tumor named after dr. This is a 48yearold man with cholangiocarcinoma klatskin with metastatic involvement of left liver lobe and left hepatic artery, also with right cardiophrenic adenopathy. His current chemotherapy regimen includes gemzar and cpt11. Best biliary drainage option in advanced klatskin tumor. The main aims of the treatment for klatskin tumor are biliary blockage breakage, resection of the tumor. During surgery they dicovered the liver was infected by 80%. Treating a klatskin tumor with three evolution controlledrelease stents in february 20, a 73yearold patient came to the st. In 73 of the resected patients 67%, all gross tumor was removed, while in 36 patients 33% gross tumor was left behind after resection. Last week my husband also 61 was diagnosed with a klatskin tumor. Hi jackie, after several echographic tests and ctscans there was still nothing visualized. Methods data on klatskin tumors between 2004 and 20 was extracted from the surveillance, epidemiology and end results registry.
After biliary ductal demonstration has been demonstrated by imaging studies, it is imperative for proximal biliary tumors that cholangiography be performed. Signs of bile duct cancer include jaundice and pain in the abdomen. Surgical resection with a tumor free margin is the only curative treatment for hilar cholangiocarcinoma klatskin tumor. Bile duct cancer is a rare disease in which malignant cancer cells form in the bile ducts. Chemotherapy chemo is treatment with cancerkilling drugs that are usually given into a vein iv or taken by mouth. Having colitis or certain liver diseases can increase the risk of bile duct cancer. The use of hormones in treatment, including somatostatin analogues, cholecystokinin, and cholecystokinin antagonists, is being investigated. The nci dictionary of cancer terms features 8,563 terms related to cancer and medicine. Definition of klatskin tumor nci dictionary of cancer. New, more aggressive surgical strategies have signi.
This network begins in the liver where many small ducts collect bile a fluid made by the liver to break down fats during digestion. The distal tumours of the common bile duct and the klatskin tumours make up the extrahe patic cca group. Of the 196 patients with klatskin tumors undergoing operative exploration, 8 4. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases. Pdf on jan 1, 2016, daniel v kostov and others published klatskin tumors find, read. Detection of klatskins tumor in extrahepatic bile duct strictures using delayed 18ffdg petct.
Demographics, tumor characteristics, treatment, and survival of. There is diffuse moderate intrahepatic bile duct dilation. Patterns of treatment failure after curative surgery show disappointingly high rates of tumor bed and regional nodal recurrence. Among this almost 60% to 70 % biliary duct cancer is klatskin tumor 1,2. In this report, we present an unusual case of 57yearold woman who has been living for more than 4. Typically, these tumors are small, poorly differentiated, exhibit aggressive biologic behavior, and tend to obstruct the intrahepatic bile ducts. Best biliary drainage option in advanced klatskin tumor the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. The ajccs tnm classification based on tumor, lymphnodes involvemen and distance metastasis 7 table 2. B, transverse image at the biliary confluence reveals an echogenic mass arrow at the bifurcation and dilated bile ducts in both lobes. Although surgical resection is the treatment of choice if possible, the prognosis is very poor after the surgery. Pdf demographics, tumor characteristics, treatment, and. Methods a population cohort study was conducted in patients selected from the surveillance, epidemiology, and. Sonographic features of these tumors include a dilatation of the intrahepatic biliary ducts but not the extrahepatic duct, b nonunion of the right and left hepatic ducts, and c small, solid masses at the hepatic hilus.
Demographics, tumor characteristics, treatment, and survival. However, over half of the patients present late with unresectable tumors. Of the 87 patients with perihilar tumors undergoing palliative procedures, 4 4. It occurs at the confluence of the right and left hepatic bile duct. However, this classification did not include lymphnode and metastasis status, and did not consider liver transplantation as option of treatment. Therapeutical concepts and results klatskkn klatskin tumors. Treatment of hilar cholangiocarcinoma klatskin tumors with hepatic resection or transplantation. The disease was named after gerald klatskin, who in 1965 described 15 cases and found some characteristics for this type of cholangiocarcinoma. A case of inoperable klatskin tumor showing response to.
The british association for the study of the liver recently published a consensus document with guidelines for the diagnosis and treatment of cholangiocarcinoma 6. Definition of klatskin tumor nci dictionary of cancer terms. Apr 07, 2020 a klatskin tumor is often diagnosed when cancer cells are seen in a liver biopsy. The authors present 3 cases of surgically proved klatskin tumor diagnosed by ultrasound alone. Survival and quality of life of cholangiocarcinoma. He is a very sportive, young looking guy, who allways lived a healthy life. A network of tubes, called ducts, connects the liver, gallbladder, and small intestine. C, the left portal vein is narrowed and encased arrows, as shown on color. Treating a klatskin tumor with three evolution controlled. Klatskin tumor genetic and rare diseases information center.
Oct, 2016 autoimmune cholangitis and primary biliary nonhodgkins lymphoma are differential diagnoses of klatskin tumors. Over 90% of extra hepatic epithelial bile duct tumors are adenocarcinomas. After a ercp they came to the conclusion that it has to be a klatskin tumor due to the location of the tissue the upper bile duct and part of the liver. Klatskin tumor is a type of cancerous growth develops at the junction between right and left biliary duct from where main bile duct is formed. Fortyfour patients in the ebd group 51% subsequently underwent ptbd. In this study, we present the epidemiology, tumor characteristics, treatment, and survival profile of klatskin tumors diagnosed between 2004 and 20. Mark fraiman on december 11, 2015 in bile cancer, bile duct cancer, bile duct disease each year approximately 15,000 new cases of liver and biliary tract cancers are diagnosed in the united states. The patient could take no solid food and complained of nausea.
Cholangiocarcinoma scandinavian journal of surgery. The only curative treatment for hilar cholangiocarcinoma is marginnegative surgical resection. Each year approximately 15,000 new cases of liver and biliary tract cancers are diagnosed in the united states. Early stage diagnosis has some options to provide curative treatment for the klatskin tumor, which include chemotherapy and radiotherapy, surgical resection of the tumor. Zentrales gallengangskarzinom klatskintumor springerlink.
Surgical resection is the mainstay for treatment of icca. Demographics, tumor characteristics, treatment, and survival of patients with klatskin tumors article pdf available in annals of gastroenterology 312 march 2018 with 298 reads. Gerald klatskin in 1965 cancer of the bile ducts can occur anywhere along the course of the biliary. The objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with hilar cholangiocarcinoma. Because klatskin tumors are often discovered after they have spread, they can be challenging to treat. Treatment results in cholangiocarcinoma are poor, with only 5% to 10% rates of overall 5year survival. Detection of klatskin s tumor in extrahepatic bile duct strictures using delayed 18ffdg petct. The primary goal of treatment and therapy for klatskin cancer is the removal of the diseased tissue and relief of biliary blockages due to tumor formations, though klatskin tumors are rare and have limited treatment options. Prognosis for the patient depends on the size of the tumor at the time of diagnosis, whether the left and right bile ducts are also involved, and the patients general physical condition. There is a high incidence of cholangiocarcinoma in patients figure 7 with ulcerative colitis 1 in 256 and primary sclerosing cholangitis 420%. Most klatskin tumours may have been coded as intra hepatic tumours for purposes of death certification. The current understanding of these tumors is limited to a small number of case reports or case series. Klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. Surgery cures the minority of patients with cholangiocarcinoma, with a 918% five year survival for proximal bile duct lesions and 2030% for distal lesions.
The patient should fully understand the prognosis of each treatment type before deciding on an option with her doctor. Having heard this klatskin name, i went online and discovered this forum. Objective klatskin tumors are rare, malignant tumors of the biliary system with a poor prognosis for patient survival. Siriwardena it started one rainy afternoon with a telephonecallfromacolleague,anattendingsurgeon at a community hospital on the periphery of our conurbation in an area of substantial social deprivation. Technical success rates 78% with ebd vs 98% with ptbd. Klatskin tumor is an extrahepatic cholangiocarcinoma cca, see this term arising in the junction of the main right or left hepatic ducts to form the common hepatic duct.
Nomograms predict survival outcome of klatskin tumors. The conclusion was that the patient had an operable klatskin tumor involving the confluence of the right anterior and posterior hepatic ducts. Apr 20, 2016 how to help a friend with a klatskin tumor diagnosis by dr. More common in native americans, mexicans, israelis, japanese.
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