I read this tumor is rare, but it sure sounds like there are lots of us out there with this type of. Klatskin tumor is a term that was traditionally given to a hilar cholangiocarcinoma, occurring at the bifurcation of the common hepatic duct. The current understanding of these tumors is limited to a small number of case reports or case series. Bile duct cancer klatskin tumor liver and pancreas surgeon. Klatskin definition of klatskin by medical dictionary. Her problems started about three weeks ago when she felt dizzy and had abdominal pain. I was diagnosed in september with a klatskin tumor it is a type of cholangio carcinoma. During surgery they dicovered the liver was infected by 80%. I have had an ultrasound, ct scan, and mrcp so far. In the left and right biliary system wires were placed.
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. Preoperative evaluation of resectability of klatskin tumor with 16mdct angiography and cholangiography original research. While there is no official known cause for klatskin tumors in the bile duct, there are some risk factors that have been associated with a small percentage of those diagnosed because they are known to cause biliary inflammation and may be related to klatskin tumor formation. Mark fraiman, md st joseph medical center liver and pancreas center 23,239 views. Klatskin tumor is quite rare, more in male sex in age 60 years. Feb 18, 2020 the median os in the klatskin tumor group was 5 months compared with 26 and 68 months in the icca and hcc groups, respectively. Oct 24, 2011 a klatskin tumor, also known as hilar cholangiocarcinoma, is a cholangiocarcinoma cancer of the biliary tree occurring at the confluence of the right and left hepatic bile ducts. But, since tumor markers can be caused by other conditions, this test isn. In 1965, klatskin identified cases of adenocarcinoma in the hepatic duct bifurcation and named this tumor type the klatskin tumor, or hilar cholangiocarcinoma hcca okuda et al.
Another classification groups biliary tract cancers as intrahepatic, perihilar and distal extrahepatic. Of the 196 patients with klatskin tumors undergoing operative exploration, 8 4. The median cancerspecific survival in the klatskin tumor group was 10 months compared with 34 months in the icca group p tumor was removed, while in 36 patients 33% gross tumor was left behind after resection. Extrahepatic biliary cystadenocarcinoma mimicking klatskin tumor.
Liver function is impaired as a result of cholestasis, and this is an important risk factor in large liver resections. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases. 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. With klatskin tumors, the cancerous cells may release certain chemicals known as tumor markers that can be detected using blood tests. 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. I will be having an ercp this friday to get a biopsy and see how much blockage there is.
Grossly classified into 3 types in some articles world j. Although surgical resection is the treatment of choice if possible, the prognosis is very poor after the surgery. Typically, these tumors are small, poorly differentiated, exhibit aggressive biologic behavior, and tend. 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. The nci dictionary of cancer terms features 8,547 terms related to cancer and medicine. Comprometimento linfonodal perihilar hepatico e do retroperitonio no estadiamento.
General rights it is not permitted to download or to forwarddistribute the text or part of it without the consent of the authors andor holders. Definition of klatskin tumor nci dictionary of cancer terms. Due to the high location of the hilar tumor an endos. Both parts were injected through a tube with contrast. All types of cancerous growth in the biliary duct are termed as cholangiocarcinoma. Mar 27, 2005 hi jackie, after several echographic tests and ctscans there was still nothing visualized. From my understanding, i thought that because of the strategic location of the klatskin tumor, it can cause obstruction and hence an earlier presentation by the patient with jaundice and thus these tmors can be resected with a much more reasonable and better prognosis compared to most cholangiocarcinomas. After extensive internet searches, im glad to find this site. Objective klatskin tumors are rare, malignant tumors of the biliary system with a poor prognosis for patient survival. Klatskin tumor is a cholangiocarcinoma in bifurcatio areas and related to poor prognosis. Recent developments in surgical techniques have improved the. In this report, we present an unusual case of 57yearold woman who has been living for more than 4. Survival and quality of life of cholangiocarcinoma patients.
The disease was named after gerald klatskin, who in 1965 described 15 cases and found some characteristics for this type of cholangiocarcinoma. Perihilar cholangiocarcinoma also known as a klatskin tumor begins in an area called the hilum, where two major bile ducts join and leave the liver. Nomograms predict survival outcome of klatskin tumors patients. B, transverse image at the biliary confluence reveals an echogenic mass arrow at the bifurcation and dilated bile ducts in both lobes. Pdf benign biliary inflammatory pseudotumor mimicking a. A case of inoperable klatskin tumor showing response to wild. Typically, these tumors are small, poorly differentiated, exhibit aggressive biologic behavior, and tend to obstruct the intrahepatic bile ducts. Benign biliary inflammatory pseudotumor mimicking a klatskin tumor.
Perihilar bile duct tumors can be further classified as type 1 through type 4, depending on the site of involvement of the ducts. Usually large, nonencapsulated, well demarcated, firm due to desmoplastic reaction, whitetan to gray and nodular intrahepatic mass. Hilar cholangiocarcinoma klatskin tumor 117 showed no pulmonary parenchymal or pleural eff usion. Kat not real name is a 64yearold indonesian lady from aceh. Endoscopic palliative therapy allowed a faster community reintegration, but with variable evolution. Tumor involving the confluence of the right and left hepatic ducts is known as klatskin tumor. Complete resection of the tumor with negative histologic margins offers the best possibility of longterm survival, and hepatic resection is a. Klatskin tumor is malignant cholangiocarcinoma arising from extrahepatic bile duct near the confluence of left and right hepatic ducts. A, longitudinal view of the common hepatic duct shows a tapered segment arrow consistent with tumor stricture. Whether an endoscopic or percutaneous transhepatic approach is favored depends on the center. Pdf demographics, tumor characteristics, treatment, and. 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. Klatskin tumor definition of klatskin tumor by the free. This paper aims to summarise the features of such klatskin like lesions that have been reported in surgical literature.
Slightly longer survival was recorded when chemotherapy was added and also after endoscopic placement of metallic stents. Any information contained in this pdf file is automatically generated from digital. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Jul 23, 20 klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. Klatskin tumor synonyms, klatskin tumor pronunciation, klatskin tumor translation, english dictionary definition of klatskin tumor. Nederlands tijdschrift voor geneeskunde, 14127, 3137.
Once the diagnosis of klatskin tumor was confirmed on cholangiogram, endoscopic stenting was performed to. Cateteres en conducto hepatico derecho e izquierdo con. A klatskin tumor or hilar cholangiocarcinoma is a cholangiocarcinoma occurring at the confluence of the right and left hepatic bile ducts. Most relevant publications describing the resection of klatskin tumors recommend preoperative decompression of the biliary duct, aiming to reduce bilirubin measurements to below 2 mgdl or 5 mgdl 2, 7, 8, 1012. Cholangiocarcinoma staging radiology reference article.
Cholangiocarcinoma of the hepatic hilum klatskin tumor. Cancer staging manual ajcc american joint committee on cancer 7ed. Hilar cholangiocarcinoma klatskin tumor scielo colombia. Klatskin tumor definition of klatskin tumor by medical. Jaundice is usually the symptom prompting a person to seek medical attention. Pdf on apr 1, 2010, dicky levenus tahapary and others published diagnosis and management of klatskin tumor. Klatskin tumor genetic and rare diseases information. Since the description by klatskin in 1965, the management of patients with adenocarcinoma of the hepatic bile duct bifurcation is viewed as a challenging clinical problem with a relatively poor prognosis. Pdf most biliary tumors at the liver hilum are malignant and, when possible, require radical. Methods a population cohort study was conducted in patients selected from the surveillance, epidemiology, and end. Therapeutical concepts and results for klatskin tumors. Any information contained in this pdf file is automatically generated from digital material submitted to epos by third parties in the form of scientific presentations. Diagnosing klatskin cancer can be hard, and you may need to have some tests done including.
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. Doppler ultrasound showed normal portal venous blood fl ow. Surgical treatment of hilar cholangiocarcinoma klatskin tumours is difficult because of its central location in the liver hilum. Quadruplephase mdct of the liver in patients with suspected hepatocellular carcinoma. Nomograms predict survival outcome of klatskin tumors. Demographics, tumor characteristics, treatment, and survival of patients with klatskin tumors. Bile duct cancer of the upper part of the bile duct is also called klatskins tumor. 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. The lack of contrast in the area of the confluence is clearly visible. Klatskin tumor symptoms typically include jaundice extreme yellowing of the skin, claycolored stools due to lack of bile drainage into the digestive system, bilirubinuria dark urine, itchy skin, weight loss, and abdominal pain. Satellite nodules are present in 30% calcification is common. We offer a widget that you can add to your website to let users look up cancerrelated terms. Klatskin tumor is an extrahepatic cholangiocarcinoma cca, see this term. Intrahepatic cholangiocarcinoma begins in the small bile ducts within the liver.
Klatskin tumor genetic and rare diseases information center. Abdominal ct scans showed neither liver compromise nor compromise of the lymph nodes, nor vascular involvement. Klatskin tumor patients than in patients with other types of tumors. Klatskin tumors occur in the hepatic duct bifurcation, usually presenting in the. Pdf diagnosis and management of klatskin tumor researchgate. This is the least common form of the disease, accounting for less than 10 percent of all cases. 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. 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. Preoperative evaluation of resectability of klatskin tumor. 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.