Stenting is used if the tumor is not possible for resection. May 17, 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. Survival and quality of life of cholangiocarcinoma. 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. This neoplasia, which originates in the epithelium of the biliary ducts, or cholangiocarcinoma cc, represents 10% of hepatobiliary tumours and 2% of malignant tumours. I am 61 years old, married and we have two wonderful grownup sons. Cholangiocarcinoma staging radiology reference article. Of the 196 patients with klatskin tumors undergoing operative. Klatskin tumor genetic and rare diseases information center. The true extent of the tumor is difficult to determine, even with highquality cholangiography. Single agent or combination chemotherapy and conventional radiation therapy are so far not. The nci dictionary of cancer terms features 8,641 terms related to cancer and medicine. He is a very sportive, young looking guy, who allways lived a healthy life.
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. Guidelines for the diagnosis and treatment of cholangiocarcinoma. Vincenzkrankenhaus in datteln, presenting with a prediagnosed klatskin tumor. Local surgical resection of hilar cholangiocarcinoma. This tumor frequently demonstrates extensive superficial spreading, resulting in diffuse involvement, which has been noted in 7% of cases of polypoid extrahepatic cholangiocarcinoma in large series,,, fig 12, 2. The current understanding of these tumors is limited to a small number. The kaplanmeier method was used to estimate overall survival os, progressionfree survival pfs, and local control lc rates, and. Surgical resection with a tumor free margin is the only curative treatment for hilar cholangiocarcinoma klatskin tumor. 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. Serum tumor markers, specifically carcinoembryonic antigen and ca199, are used for the diagnosis, treatment, and monitoring of hc with 89% sensitivity and 86% specificity when combined with other.
Jul 23, 20 klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. In this study, we present the epidemiology, tumor characteristics, treatment, and survival profile of klatskin tumors diagnosed between 2004 and 20. 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. Cholangiocarcinomas are classified by the international classification of diseases for oncology icdo according to their anatomic site topography and histology morphology 4. Definition of klatskin tumor nci dictionary of cancer terms. The 5year survival after surgical treatment with curative intention is 1030%, but at the time of diagnosis less than onethird of tumors are resectable3,4. Although surgical resection is the treatment of choice if possible, the prognosis is very poor after the surgery.
However, there has been rare established as definite management of cholecystitis instead of interval cholecystectomy especially in patient with unresectable klatskin tumor. Clinical outcomes and toxicity of proton beam therapy for. Klatskin s tumor is a common cause of malignant biliary obstruction5. Dec 11, 2015 a closer look at klatskin tumor treatment options by dr. Surgery cures the minority of patients with cholangiocarcinoma, with a 918% five year survival for proximal bile duct lesions and 2030% for distal lesions. Having heard this klatskin name, i went online and discovered this forum. Because of the absence of early symptoms, klatskins tumor is often diagnosed at an advanced stage, when jaundice is the most important clinical symptom2.
It is not clear, however, whether similar results can be reproduced in a group of patients with klatskin tumor. Pdf on jan 1, 2016, daniel v kostov published klatskin tumors find, read and cite all. Jan 14, 2014 we examined the efficacy and toxicity of proton beam therapy pbt for treating advanced cholangiocarcinoma. We further evaluate differences between extra and intrahepatic klatskin tumors, as prior reports suggest that they tend to present and behave differently. Nomograms predict survival outcome of klatskin tumors. The patient could take no solid food and complained of nausea. Palliation of patients with mhs is a difficult clinical problem with little consensus regarding the optimal treatment approach. Curative treatment of klatskin tumors by radical surgical procedures with surgical preparation distant to the tumor region results in 5year survival rates of 3050%. Medical treatment of ccc involves any of the following. Klatskin tumor is quite rare, more in male sex in age 60 years.
Of the 196 patients with klatskin tumors undergoing operative exploration, 8 4. Treating a klatskin tumor with three evolution controlledrelease stents in february 20, a 73yearold patient came to the st. Endoscopic retrograde cholangiopancreatography and endoscopic. The use of hormones in treatment, including somatostatin analogues, cholecystokinin, and cholecystokinin antagonists, is being investigated. 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 the ability of the tumor. It is best evaluated by ultrasound prior to placement of biliary drainage catheters, as pneumobilia or stent artifact may obscure tumor. In this report, we present an unusual case of 57yearold woman who has been living for more than 4. What is the survival rate for nonresectable klatskin tumor. It is estimated that 23,000 cases of cholangiocarcinoma are diagnosed in japan every year, an incidence higher than that in western countries. These tumors tend to become symptomatic late in their development and therefore.
Demographics, tumor characteristics, treatment, and. Clinically, jaundice and pruritus are the most common symptoms at diagnosis. The overall picture was consistent with a presentation of klatskin tumor a representative picture of which, from an unrelated patient, is shown in figure 2b, given the elevated ca199 level. The current understanding of these tumors is limited to a small number of case reports or case series. N2 cancer of the biliary tree, including those occurring at the major biliary bifurcation klatskin s tumor, is an uncommon malignancy. Gerald klatskin in 1965 cancer of the bile ducts can occur anywhere along the course of the biliary.
The most recent 2017 revision of the tumor, node, metastasis tnm classification no longer considers type iv tumors to represent a t4 primary tumor. Treatment ofhilar cholangiocarcinoma klatskin tumors with. Abstract background the objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with hilar cholangiocarcinoma. Methods a population cohort study was conducted in patients selected from the surveillance, epidemiology, and end. 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. Cholangiocarcinomas, primary bile duct cancers, are rare but highly fatal tumors 1 3. Cholangiocarcinomas are classified by the international classification of diseases for oncology icd. Hi jackie, after several echographic tests and ctscans there was still nothing visualized. 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. 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. Subsequently, a series of patients with this diagnosis was reported by klatskin in 1965. Treatment ofhilar cholangiocarcinoma klatskin tumors. The disease was named after gerald klatskin, who in 1965 described 15 cases and found some characteristics for this type of 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. Klatskin tumor, a hilar cholangiocarcinoma of the biliary tree occurs at the confluence of the right and left hepatic bile ducts. Recent reports with increasing numbers of patients have allowed the construction of rational approaches to these patients. Treatment of hilar cholangiocarcinoma klatskin tumors with. Last week my husband also 61 was diagnosed with a klatskin tumor. Prognosis for the patient depends on the size of the tumor. Klatskins tumor is a common cause of malignant biliary obstruction5. 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. Klatskin tumor genetic and rare diseases information. Furthermore, the incidence and mortality from cholangiocarcinoma are increasing worldwide6,7.
Because of the absence of early symptoms, klatskin s tumor. The clinical data and outcomes of 28 cholangiocarcinoma patients treated with pbt between january 2009 and august 2011 were retrospectively examined. This requires mandatory en bloc liver resection and resection of the extrahepatic bile duct often together with vascular resection. Carcinoma of the hepatic duct bifurcation was first described by altemeir in 1957. Best biliary drainage option in advanced klatskin tumor. Vincenzkrankenhaus in datteln, presenting with a prediagnosed klatskintumor bismuth iv with worsening general condition. It has been suggested that preoperative endoscopic. However, over half of the patients present late with unresectable tumors. In 73 of the resected patients 67%, all gross tumor was removed, while in 36 patients 33% gross tumor was left behind after resection. Endoscopic endoprosthesis insertion as a palliative treatment has been reported 1,2 to have a highly successful drainage rate in patients with malignant hilar biliary obstruction. Endoscopic retrograde cholangiopancreatography and endoscopic endoprosthesis insertion have a limited value in the management of patients with klatskin tumors. Perihilar cholangiocarcinoma, also called klatskin tumor, can be defined as a tumor located above the junction of the cystic duct up to and including the secondorder biliary branches of the right and left.
In recent decades, surgical treatment of hilar cholangiocarcinoma has moved toward. Unresectable hilar cholangiocarcinoma klatskin tumor is a rare malignancy with limited treatment options and an overall poor. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases. There is prooved connection between this malignant tumor and primary sclerosis cholangitis autoimmune disease and parasitic infection. Each year approximately 15,000 new cases of liver and biliary tract cancers are diagnosed in the united states. 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 the ability of the tumor to infiltrate longitudinally and radially along the biliary tree necessitates aggressive resection strategies such as ipsilateral hepatectomy. Apr 20, 2016 how to help a friend with a klatskin tumor diagnosis by dr. Impact of classification of hilar cholangiocarcinomas. Novel therapy for unresectable hilar cholangiocarcinoma and. Optimal biliary drainage for inoperable klatskins tumor.
Treating a klatskin tumor with three evolution controlled. Although surgery is the only potentially curative treatment. A klatskin tumor is a tumor which forms in the upper region of the bile duct, where the left and right bile ducts meet. Jun 21, 2006 cholangiocarcinomas, primary bile duct cancers, are rare but highly fatal tumors 1 3. During surgery they dicovered the liver was infected by 80%.
Klatskin tumor treated by interdisciplinary therapies. Chemotherapy versus surgery in primary bcell lymphoma masquerading as klatskin tumor a diagnostic and therapeutic dilemma. Objective klatskin tumors are rare, malignant tumors of the biliary system with a poor prognosis for patient survival. Apr 20, 2020 the use of hormones in treatment, including somatostatin analogues, cholecystokinin, and cholecystokinin antagonists, is being investigated. 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. P110efficacy of percutaneous transhepatic gallbladder. A case of inoperable klatskin tumor showing response to. Efficacy of percutaneous transhepatic gallbladder drainage. It occurs at the confluence of the right and left hepatic bile duct. We offer a widget that you can add to your website to let users look up cancerrelated terms. Manual crossreferencing was performed and relevant references from. To continue reading this article, you must log in with your personal, hospital, or group practice subscription.
Because of the rarity of this malignancy, its prognostic risk factors have not been completely analyzed. How to help a friend with a klatskin tumor diagnosis. Demographics, tumor characteristics, treatment, and survival of patients with klatskin tumors article pdf available in annals of gastroenterology 312 march 2018 with 298 reads. Leading doctors for treatments of klatskin tumors doctor. Tumors involving the hepatic duct bifurcation hilar cholangiocarcinomas are collectively referred to as klatskin tumors. Cholangiocarcinoma ccc is the second most common primary liver cancer after hepatocellular carcinoma. 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.
Survival rates for 1,3, and 5 years after hepatic resection were 74%, 34%, and 9%, respectively, and. Cholangiocarcinoma as a form of cancer is incurable and can be deadly, unless all the malignant tumors are surgically resected or totally removed. Klatskin tumor is a term that was traditionally given to a hilar cholangiocarcinoma, occurring at the bifurcation of the common hepatic duct. Cholangiocarcinoma survival rate, prognosis, foundation. Since that time, cholangiocarcinomas at this location have carried the eponym of klatskin tumors. 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. Percutaneous transhepatic gallbladder drainage ptgbd is an effective treatment for cholecystitis in patients with poor general condition. Cholangiocarcinoma originally referred only to primary tumours of the intrahepatic bile ducts and was not used for extrahepatic. After a ercp they came to the conclusion that it has to be a klatskin tumor due to the location of the. Cancer of the biliary tree, including those occurring at the major biliary bifurcation klatskin s tumor, is an uncommon malignancy. Generally, if they demonstrate a focal stenosis of the common hepatic duct, a presumptive diagnosis of klatskin tumor is made 2. Chemotherapy versus surgery in primary bcell lymphoma masquerading as klatskin tumor. Type iv tumors, defined as tumor invasion of the second order biliary radicles.
Listing a study does not mean it has been evaluated by the u. In the case of unresectable tumors, then survival rate is minimum, whereas 25% of 30% surgical cases can survive up to five years 6. Symptoms usually dont present until advanced stages of disease, when jaundice is the most common feature. Pdf demographics, tumor characteristics, treatment, and. How to help a friend with a klatskin tumor diagnosis by dr. Mar 27, 2005 hi jackie, after several echographic tests and ctscans there was still nothing visualized. Endoscopic retrograde cholangiopancreatography and. Treatment of hilar cholangiocarcinoma klatskin tumors. Pdf diagnosis and management of klatskin tumor researchgate. 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.
Endoscopic or percutaneous biliary drainage for klatskin. Hann, in blumgarts surgery of the liver, pancreas and biliary tract fifth edition, 2012. The survival rate of klatskin tumor is poorer as these tumors are only diagnosed at an advanced stage. This manuscript has been developed with the support of the british liver. Cholangiocarcinoma is a malignant tumor arising from the epithelium of the bile ducts. Demographics, tumor characteristics, treatment, and survival. 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. A case of inoperable klatskin tumor showing response to wild. Methods data on klatskin tumors between 2004 and 20 was extracted from the surveillance, epidemiology and end results registry. Hilar cholangiocarcinoma or klatskin tumor see chapter 50c, occurs at the biliary confluence. Detection of benign hilar bile duct stenosesa retrospective analysis.