Hilar cholangiocarcinoma grows in a narrow and pivotal location, surrounded by. Stenting is used if the tumor is not possible for resection. Oct, 2016 autoimmune cholangitis and primary biliary nonhodgkins lymphoma are differential diagnoses of klatskin tumors. Apr 07, 2020 a klatskin tumor is a tumor which forms in the upper region of the bile duct, where the left and right bile ducts meet. Guidelines for the diagnosis and treatment of cholangiocarcinoma. Pdf nomograms predict survival outcome of klatskin tumors. Survival rates for 1,3, and 5 years after hepatic resection were 74%, 34%, and 9%, respectively, and those after transplantation were 60%, 32%, and 25%, respectively. Pathology outlines carcinoma of extrahepatic bile ducts.
Methods data on klatskin tumors between 2004 and 20 was extracted from the surveillance, epidemiology and end results registry. Nomograms predict survival outcome of klatskin tumors patients. When tumors are deemed unresectablemake sure a surgical oncologist skilled in hepatic. Curative treatment of klatskin tumors by radical surgical procedures with surgical preparation distant to the tumor region results in 5year survival rates of 3050%. Klatskin tumor is quite rare, more in male sex in age 60 years. Although surgical resection is the treatment of choice if possible, the prognosis is very.
Treating a klatskin tumor with three evolution controlled. It was his dream to create a histological atlas of diseases of the liver, an ambition brought to fruition in 1993 by harold conn, his first fellow and longtime collaborator at yale. Therapeutical concepts and results for klatskin tumors ncbi. Met when all patients died before the treatment became available but some now. Cholangiocarcinoma as a form of cancer is incurable and can be deadly, unless all the malignant tumors are surgically resected or totally removed. However, over half of the patients present late with unresectable tumors. The content on the uptodate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment.
Listing a study does not mean it has been evaluated by the u. The patient could take no solid food and complained of nausea. 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. Best biliary drainage option in advanced klatskin tumor. Klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. Cholangiocarcinoma staging is dependent on whether the tumor is primarily intrahepatic icc, hilarperihilar klatskin, or extrahepatic tumor resection is currently the most optimal treatment and. Overall survival os was influenced by summary stage, lymph node metastases, and surgery. Another classification groups biliary tract cancers as intrahepatic, perihilar and distal extrahepatic.
I am trying to be very positive about it but truthfully its driving me crazy, every little pain i get i think the worse. This section provides resources to help you learn about medical research and ways. The current understanding of these tumors is limited to a small number of case reports or case series. Klatskin tumors are rare, malignant tumors of the biliary system with a poor prognosis for patient survival. After extensive internet searches, im glad to find this site. 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.
Surgical treatment of hilar cholangiocarcinoma hcca has shifted from local excision of the bile duct confluence at the liver hilum during the. Cholangiocarcinoma staging radiology reference article. 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. Recent developments in surgical techniques have improved the. Her problems started about three weeks ago when she felt dizzy and had abdominal pain. A case of inoperable klatskin tumor showing response to. Hilar cholangiocarcinoma, also known as klatskin tumour, is the commonest type of cholangiocarcinoma. Klatskin tumors are anatomically defined as extrahepatic cholangiocarcinomas that occur at the confluence of the left and right hepatic duct fig. Demographics, tumor characteristics, treatment, and.
A new clinical classification of hilar cholangiocarcinoma. Mark fraiman on april 20, 2016 in bile cancer, bile duct cancer if someone close to you, say a family member or friend, has. Early stage diagnosis has some options to provide curative treatment for the klatskin tumor, which include chemotherapy and radiotherapy, surgical resection of the tumor. I will be having an ercp this friday to get a biopsy and see how much blockage there is. Research helps us better understand diseases and can lead to advances in diagnosis and treatment. Local extension occurs into the liver, porta hepatis, and regional lymph nodes of the celiac and pancreaticoduodenal chains. Presence of intraepithelial spread is not an indicator of a poor prognosis but carcinoma in situ at the bile duct stump can cause recurrence mod pathol 2008. Demographics, tumor characteristics, treatment, and survival of. Among these tumors, extrahepatic and surgically treated tumors tend to have better outcomes. Preoperative evaluation of resectability of klatskin tumor. Definition of klatskin tumor nci dictionary of cancer. In 73 of the resected patients 67%, all gross tumor was removed, while in 36 patients 33% gross tumor was left behind after resection. The nci dictionary of cancer terms features 8,563 terms related to cancer and medicine we offer a widget that you can add to your website to let users look up cancerrelated terms. Because klatskin tumors are often discovered after they have spread, they can be challenging to treat.
Clinical outcomes and toxicity of proton beam therapy for. The use of hormones in treatment, including somatostatin analogues, cholecystokinin, and cholecystokinin antagonists, is being investigated. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases. Hilar cholangiocarcinoma altemeierklatskin tumor, is an uncommon malignant neoplasm, arising from the bile duct epithelium of the common hepatic duct or its first and second bifurcation. 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. Demographics, tumor characteristics, treatment, and survival of patients with klatskin tumors article pdf available in annals of gastroenterology 312 march 2018 with 270 reads. How to help a friend with a klatskin tumor diagnosis. Tumors involving the hepatic duct bifurcation hilar cholangiocarcinomas are collectively referred to as klatskin tumors. What signs and symptoms may be associated with klatskin tumors.
Mar 27, 2005 my lymph nodes were not affected and the margin of the liver came back negative. Treatment ofhilar cholangiocarcinoma klatskin tumors. Treatment of hilar cholangiocarcinoma klatskin tumors with. Subsequently, a series of patients with this diagnosis was reported by klatskin in 1965. Kat not real name is a 64yearold indonesian lady from aceh. Klatskin tumor or hilar cholangiocarcinoma is a cholangiocarcinoma which.
Surgical treatment of hilar cholangiocarcinoma klatskin tumours is difficult because of its central location in the liver hilum. Survival and quality of life of cholangiocarcinoma patients. The best treatment for a klatskin tumor is resection, in which the tumor is removed, with clean margins to remove any malignant cells which may be associated with the klatskin tumor. Cholangiocarcinoma of the hepatic duct confluence klatskin tumor is an uncommon malignancy associated with poor prognosis and a high mortality rate. The patients receiving metallic stents had better survival than those receiving plastic stents 40. Jul 23, 20 klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. Extent of liver resection for hilar cholangiocarcinoma klatskin. Since that time, hilar cholangiocarcinomas at this location have carried the eponym of klatskin tumors. Perihilar bile duct tumors can be further classified as type 1 through type 4, depending on the site of involvement of the ducts. My lymph nodes were not affected and the margin of the liver came back negative. There is no established standard palliative chemotherapy.
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. Visit the orphanet disease page for more information. Article pdf available in world journal of surgery 3911 july 2015 with 331 reads how we measure reads. Therapeutical concepts and results klatskkn klatskin tumors. Lifethreatening infection cholangitis may occur that requires immediate antibiotic intervention and. This neoplasia, which originates in the epithelium of the biliary ducts, or cholangiocarcinoma cc, represents 10% of hepatobiliary tumours and 2% of malignant tumours. A new clinical classification of hilar cholangiocarcinoma klatskin tumor. In recent years, with the invention and development of the technology of mdct, increasing numbers of reports have been published on its use for preoperative assessment of liver tumors and pancreatic. The documents contained in this web site are presented for information purposes only.
Sonographic features of these tumors include a dilatation of the intrahepatic biliary ducts but not the. Klatskin tumor genetic and rare diseases information. Hilar cholangiocarcinoma altemeierklatskin tumor 1, 2 is an uncommon malignant neoplasm, arising from the bile duct epithelium of the common hepatic duct or its first and second bifurcation. Of the 196 patients with klatskin tumors undergoing operative exploration, 8 4. The main types of treatments for klatskin tumors are. The clinical data and outcomes of 28 cholangiocarcinoma patients treated with. There is prooved connection between this malignant tumor and primary sclerosis cholangitis autoimmune disease and parasitic infection in.
Jan 14, 2014 we examined the efficacy and toxicity of proton beam therapy pbt for treating advanced cholangiocarcinoma. Surgical resection with a tumorfree margin is the only curative treatment for hilar cholangiocarcinoma klatskin tumor. Klatskin tumors are cholangiocarcinomas that develop at the point where the left and right hepatic ducts join to form the common hepatic duct. Pdf on jan 1, 2016, daniel v kostov and others published klatskin tumors find, read. The clinical data and outcomes of 28 cholangiocarcinoma patients treated with pbt between january 2009 and august 2011 were retrospectively examined. Cholangiocarcinoma accounts for less than 2% of all human malignancies but is the second most common primary liver tumor 1. Vincenzkrankenhaus in datteln, presenting with a prediagnosed klatskintumor bismuth iv with worsening general condition. In this report, we present an unusual case of 57yearold woman who has been living for more than 4.
Treatment ofhilar cholangiocarcinoma klatskin tumors with hepatic resection or transplantation shunzaburo iwatsuki, md, rho. Treating a klatskin tumor with three evolution controlledrelease stents in february 20, a 73yearold patient came to the st. Klatskin tumor, ercp, stent, diagnosis, management. I am 61 years old, married and we have two wonderful grownup sons. Of the 196 patients with klatskin tumors undergoing operative. Surgical removal of the tumor and relief of bile duct blockage are the main goals of treatment. Targeted therapy for extrahepatic klatskin tumor cc. What symptoms are common in patients with nonresectable klatskin tumors. If im able to get rid of the vest in the next 3 weeks, ill be headed to sloankettering in nyc to look for any treatment that i can tolerate. The kaplanmeier method was used to estimate overall survival os, progressionfree survival pfs, and local control lc rates, and.
Apr 20, 2020 cholangiocarcinomas tend to grow slowly and to infiltrate the walls of the ducts, dissecting along tissue planes. We examined the efficacy and toxicity of proton beam therapy pbt for treating advanced cholangiocarcinoma. There are a number of inflammatory, infective, vascular, and other pathologies, which have overlapping clinical and radiological features with a klatskin tumour, leading to misinterpretation. My doctor said there is no treatment for a klatskin tumor. 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 term cholangiocarcinoma includes all bile duct cancers. It has been suggested that preoperative endoscopic. One and 5year causespecific survival for klatskin tumors was 41% and 10.
Hilar cholangiocarcinoma altemeier klatskin tumor 1, 2 is an uncommon malignant neoplasm, arising from the bile duct epithelium of the common hepatic duct or its first and second bifurcation. Last week my husband also 61 was diagnosed with a klatskin tumor. Apparently, this cardiac side effect of 5fu is pretty rare. Hilar cholangiocarcinoma klatskin tumor gerald klatskin 19101986 adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis 1965 the american journal of medicine. Demographics, tumor characteristics, treatment, and survival.
Medical treatment of ccc involves any of the following. Klatskin tumor genetic and rare diseases information center. Pdf diagnosis and management of klatskin tumor researchgate. Frozen section analysis, hilar cholangiocarcinoma, klatskin tumor, prognosis. A case of inoperable klatskin tumor showing response to wild. 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. Cholangiocarcinoma survival rate, prognosis, foundation. Although surgical resection is the treatment of choice if possible, the prognosis is very poor after the surgery. I have had an ultrasound, ct scan, and mrcp so far. We offer a widget that you can add to your website to let users look up cancerrelated terms. A, longitudinal view of the common hepatic duct shows a tapered segment arrow consistent with tumor stricture. If you have problems viewing pdf files, download the latest version of adobe reader.
Original article impact of different tumor size cutoff points on. Tumor involving the confluence of the right and left hepatic ducts is known as klatskin tumor. Siriwardena it started one rainy afternoon with a telephonecallfromacolleague,anattendingsurgeon at a community hospital on the periphery of our. I was diagnosed in september with a klatskin tumor it is a type of cholangio carcinoma. Pdf demographics, tumor characteristics, treatment, and. But, if its caught early enough, there is a good chance that it can be cured completely. 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. Quadruplephase mdct of the liver in patients with suspected hepatocellular carcinoma. The authors present 3 cases of surgically proved klatskin tumor diagnosed by ultrasound alone. New, more aggressive surgical strategies have signi. 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. Apr 20, 2020 the use of hormones in treatment, including somatostatin analogues, cholecystokinin, and cholecystokinin antagonists, is being investigated. Department of hepatopancreaticobiliary surgery and liver. Surgical procedure and longterm survival of hilar cholangiocarcinoma.
In 1965 he described the unique features of cholangiocarcinoma at the bifurcation of the main intrahepatic bile ducts, which came to be called the klatskin tumor. The prognosis of klatskin tumors was closely related to age, lymph node status, summary stage, and local treatment of the primary tumor according to multivariate regression analysis. Nomograms predict survival outcome of klatskin tumors. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. It poses unique problems in the diagnosis and management because of its. Preoperative evaluation of resectability of klatskin tumor with 16mdct angiography and cholangiography original research.
Doctor answers on symptoms, diagnosis, treatment, and more. 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. The current understanding of these tumors is limited to a small number. Perihilar bile duct tumors can be further classified as type 1 through type 4, depending on the site of. How to help a friend with a klatskin tumor diagnosis by dr. I read this tumor is rare, but it sure sounds like there are lots of us out there with this type of.
Dec 11, 2015 treatment options for klatskin tumors. The nci dictionary of cancer terms features 8,563 terms related to cancer and medicine. Treatment ofhilar cholangiocarcinoma klatskin tumors with. Extrahepatic bile ducts carcinoma of extrahepatic bile ducts. Klatskin tumor treatment is usually aimed at controlling the symptoms of cancer for as long as possible. 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. Surgical strategies in the management of hilar cholangiocarcinoma.
They are malignant tumors that were first described in 1965. Definition of klatskin tumor nci dictionary of cancer terms. B, transverse image at the biliary confluence reveals an echogenic mass arrow at the bifurcation and dilated bile ducts in both lobes. My doctor said the chances of the cancer coming back is 5050. The main aims of the treatment for klatskin tumor are biliary blockage breakage, resection of the tumor. The prognosis of patients with klatskin tumors has been significantly. Type iv tumors, defined as tumor invasion of the second order biliary radicles bilaterally, are associated with a higher rate of positive surgical margins and significantly poorer overall survival after resection compared with types i to iii 6. The disease was named after gerald klatskin, who in 1965 described 15 cases and found some characteristics for this type of cholangiocarcinoma. This paper aims to summarise the features of such klatskin like lesions that have been reported in surgical literature. Objective klatskin tumors are rare, malignant tumors of the biliary system with a poor prognosis for patient survival. Abstract background the objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with hilar cholangiocarcinoma. Klatskin tumor is an extrahepatic cholangiocarcinoma cca, see this term arising in the junction of. Treatment of hilar cholangiocarcinoma klatskin tumors. Klatskin tumor is a term that was traditionally given to a hilar cholangiocarcinoma, occurring at the bifurcation of the common hepatic duct.
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