Klatskin tumors are a type of cancer located in the upper part of the bile duct affecting the right or left hepatic ducts as they enter the liver. Klatskin tumor patients than in patients with other types of tumors. Prise en charge chirurgicale du cholangiocarcinome hilaire. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases. Klatskin tumor synonyms, klatskin tumor pronunciation, klatskin tumor translation, english dictionary definition of klatskin tumor. Stereotactic body radiotherapy sbrt for intrahepatic and hilar cholangiocarcinoma. Complete resection of the tumor with negative histologic margins offers the best possibility of longterm survival, and hepatic resection is a. A case of inoperable klatskin tumor showing response to wild. Unfortunately, most hc patients are diagnosed at later stages, when the tumor has.
Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever. 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. Hilar cholangiocarcinoma hc, also known as klatskin tumor, is a complex and devastating malignancy of the bile duct. A, longitudinal view of the common hepatic duct shows a tapered segment arrow consistent with tumor stricture. Free fulltext pdf articles from hundreds of disciplines, all in one place percutaneous irreversible electroporation of unresectable hilar cholangiocarcinoma klatskin tumor. In 90% of cases klatskin tumors occur sporadically but certain risk factors have been associated with the disease. The system is intended to provide practice to physicians, fellows and residents in the interpreptation of radiological images from a variety of different cases. Endoscopic palliative therapy allowed a faster community reintegration, but with variable evolution. Mimics of malignancy in abdominal imaging michael p hartung, md1 christine o menias, md2 vincent m mellnick, md3 meghan g lubner, md1 perry j pickhardt, md1 1department of radiology, university of wisconsinmadison, madison, wi. I will be having an ercp this friday to get a biopsy and see how much blockage there is. Among this almost 60% to 70 % biliary duct cancer is klatskin tumor 1,2. Hilar cholangiocarcinoma klatskin tumor 117 showed no pulmonary parenchymal or pleural eff usion.
Traitement chirurgical des cholangiocarcinomes perihilaires fmc. Cholangiocarcinoma, a spectrum of intrahepatic, perihilar and distal tumors. The difficulties with the classification of klatskin tumor pose a problem of an adequate assessment of the incidence 3 although it is known that klatskin tumors account for 67% of all ccs 1. Ercp dilemma most accurate bile duct visualization but technically demanding filling of all.
Bile duct cancer klatskin tumor liver and pancreas surgeon. Classification du mskcc memorial sloankettering cancer center. Her problems started about three weeks ago when she felt dizzy and had abdominal pain. B, transverse image at the biliary confluence reveals an echogenic mass arrow at the bifurcation and dilated bile ducts in both lobes. Sclerosing carcinoma of the major intrahepatic bile ducts. Klatskin tumor, or hilar cca involving the bifurcation of the hepatic duct, accounts for approximately 60 % of all bile duct cancers and is the second most common primary malignant hepatobiliary neoplasm. Klatskin tumor is a term that was traditionally given to a hilar cholangiocarcinoma, occurring at the bifurcation of the common hepatic duct. Gallbladder cancer with tumor thrombus in the portal vein. Chemotherapy versus surgery in primary bcell lymphoma masquerading as klatskin tumor a diagnostic and therapeutic dilemma. Cholangiocarcinoma is a malignant neoplasm of the hepatic bile. Mesohepatectomy, caudate lobectomy and resection of hilar bifurcation with biliary reconstruction by 6 hepaticojejunostomies for klatskin tumor. Treatment ofhilar cholangiocarcinoma klatskin tumors with.
Klatskin tumor genetic and rare diseases information. Cholangiocarcinoma of the hepatic hilum klatskin tumor. Surgical resection is the most effective management for hc and offers a relatively longterm survival when performed during early stages of cancer. Perihilar bile duct tumors can be further classified as type 1 through type 4, depending on the site of involvement of the ducts. This paper aims to summarise the features of such klatskin like lesions that have been reported in surgical literature. Chemotherapy versus surgery in primary bcell lymphoma masquerading as klatskin tumora diagnostic and therapeutic dilemma. Tumor extent metastases volumetry left side klatskin. 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. Department of radiation oncology, harvard medical school, boston. 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. Patient detail from the seer database with diagnosed liver and intrahepatic bile duct cancer from 2004 to 2014 each variable represents the details of the diagnosis of liver and and intrahepatic bile duct cancer, such as insurance, diagnostic age, race, gender, stage, grade, tumor size, lymph node status, and survival time.
Gallbladder cancer gbc is a highly fatal disease, has a low response to treatment, and a poor overall prognosis, due to detection at a late stage. 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. Surgery using extended liver resections with an en bloc resection of the liver with. Full text full text is available as a scanned copy of the original print version. Local resection of biliopancreatic cancer annals of. 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.
Anand mahadevan 1, nergiz dagoglu 1, joseph mancias 1, kristin raven 2, khalid khwaja 2, jennifer f tseng 2, kimmie ng 3, peter enzinger 3, rebecca miksad 4, andrea bullock 4, amy evenson 2. Klatskin tumors usually show signs of biliary obstruction with jaundice and pale stools 6. Typically, these tumors are small, poorly differentiated, exhibit aggressive biologic behavior, and tend. Percutaneous irreversible electroporation of unresectable. Kat not real name is a 64yearold indonesian lady from aceh. Mar 27, 2005 hi jackie, after several echographic tests and ctscans there was still nothing visualized. A klatskin tum or or hila r cholangiocarcino ma is a cholangiocarcinoma can cer of the biliary tree occurring at the confluence of the right and left hepatic bile ducts. Another classification groups biliary tract cancers as intrahepatic, perihilar and distal extrahepatic. Comprometimento linfonodal perihilar hepatico e do retroperitonio no estadiamento.
Aug 20, 2010 hilar cholangiocarcinoma is a primary cancer of the bile ducts located at the hilar bifurcation. Origin and pathological characteristics of klatskin tumor. After extensive internet searches, im glad to find this site. Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of vater. Radiology teaching files university of rochester medical. Neuroendocrine tumor net arising from common bile duct cbd is a rare disease entity, whose origin is currently in discussion with various theories. Differential diagnosis in metastatic adenocarcinoma of the liver a case report 27 februarie 2019. Bevacizumab, in combination with 5fluorouracilbased chemotherapy, is indicated for first and secondline treatment of metastatic colorectal adenokarinoma. Pubmed is a searchable database of medical literature and lists journal articles that discuss klatskin tumor. Cholangiocarcinoma involving the hepatic bifurcation are categorized as hilar bile duct cancer or klatskin tumors. Stereotactic body radiotherapy sbrt for intrahepatic and. Jaundice is usually the symptom prompting a person to seek medical attention. Klatskin tumors, or hilar ccas, are bile duct tumors that involve the common hepatic duct bifurcation but may arise from the intrahepatic ihcca. Slightly longer survival was recorded when chemotherapy was added and also after endoscopic placement of metallic stents.
The disease was named after gerald klatskin, who in 1965 described 15 cases and found some characteristics for this type of cholangiocarcinoma. As far as the extrafollicular variant, we agree with philipsen, et al. Jul 23, 20 klatskin tumor is known to be highly resistant to conventional chemotherapy and radiotherapy. 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. Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts. The nci dictionary of cancer terms features 8,547 terms related to cancer and medicine.
Cateteres en conducto hepatico derecho e izquierdo con. Early detection and diagnosis were associated with a higher rate of os in. Klatskin tumor genetic and rare diseases information center. In this report, we present an unusual case of 57yearold woman who has been living for more than 4.
According to the proximal tumor extension the hilar cholangiocarcinoma is classified by bismuthcorlette. I read this tumor is rare, but it sure sounds like there are lots of us out there with this type of. Abdominal ct scans showed neither liver compromise nor compromise of the lymph nodes, nor vascular involvement. Definition of klatskin tumor nci dictionary of cancer terms. Management of hilar cholangiocarcinoma in the north of england. I have had an ultrasound, ct scan, and mrcp so far. Tumor involving the confluence of the right and left hepatic ducts is known as klatskin tumor.
The full text of this article is available in pdf format. In the ideal animal model of cca, the tumor arises from the biliary tract in an immunocompetent host with a speciesmatched tumor microenvironment. Role of fractionated external beam radiotherapy in hemangioblastoma of the central nervous system. From 19831997, 112 patients underwent surgical resection of a carcinoma of the hepatic duct sonfluence klatskin tumor. Klatskin tumor is an extrahepatic cholangiocarcinoma cca, see this term arising in the. Although surgical resection is the treatment of choice if possible, the prognosis is very poor after the surgery. Diagnosis of biliary strictures ercp and tissue diagnosis. It is the most common malignant tumor of the bile duct system and the fifth most common malignant. Neuroendocrine tumor arising from common bile duct.
We present a case of welldefined solid, exophytic and arterial hypervascular mass arising from cbd with relatively mild dilated central intrahepatic bile ducts in 50yearold man, which was surgically confirmed to be extrahepatic. All types of cancerous growth in the biliary duct are termed as cholangiocarcinoma. It is quite a rare malignant tumor yearly incidence of 24 out of 100,000 patients, but nevertheless the fourth most common. Survival and quality of life of cholangiocarcinoma patients. Mark fraiman, md st joseph medical center liver and pancreas center 23,239 views. In 73 of the resected patients 67%, all gross tumor was removed, while in 36 patients 33% gross tumor was left behind after resection. Radiation oncologybile ductadjuvant rt wikibooks, open. First, ctvts can ttansmisible be experimentally induced by transplanting living vvenereo cells, and tumor venereo transmisible en perros by killed cells tumor venereo transmisible en perros cell filtrates. Get a printable copy pdf file of the complete article 499k, or click on a page image below to browse page by page. International classification of diseases for oncology. Pdf demographics, tumor characteristics, treatment, and.
Cholangiocarcinoma staging radiology reference article. Ainsi, lorsquil est negatif, le brossage nexclut pas le cancer. Pfeifferimpact of classification of hilar cholangiocarcinomas klatskin tumors on the incidence of intra and extrahepatic cholangiocarcinoma in the. 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. 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. Cholangiocarcinomas cccs are malignancies of the biliary duct system perihilar tumors, also called klatskin tumors after klatskin s description of them in the etiology of most bile duct cancers remains undetermined. Bile duct system papilla of vater standards and innovations diagnostic why. Local resection was undertaken in 80 patients 52 patients with type i and ii tumors, and 28 patients with type iii tumors whereas in 32 patients with type ie tumors, hilar resection was performed with liver resection. In addition to the liver, the composite allografts included various combina. Demographics, tumor characteristics, treatment, and survival of patients with klatskin tumors.
Genomic dna was extracted and served as a template for pcr amplification and sequencing. Klatskin tumor principles of drainage to relieve jaundice, only a minority of liver 25 %. Of the 196 patients with klatskin tumors undergoing operative exploration, 8 4. Doppler ultrasound showed normal portal venous blood fl ow. Klatskin tumor is quite rare, more in male sex in age 60 years. Third admission in july,examination showed a tense suboccipital decompression, blindness on the right due to a completely detached retina, a superior nasal defect in the left field of vision with a normal left ocular fundus, dysarthria, and ataxia of both arms. Klatskin tumor definition of klatskin tumor by the free. Preoperative biliary drainage increases infectious complications after hepatectomy for proximal bile duct tumor obstruction. Hilar cholangiocarcinoma, sometimes referred to as a klatskin tumor, is a rare neoplasm arising proximal to the confluence of the cystic and common hepatic ducts. 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.
Due to the high location of the hilar tumor an endos. Demographics, tumor characteristics, treatment, and survival of. 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. We offer a widget that you can add to your website to let users look up cancerrelated terms. This stage adenomayoid called protective stage of amelogenesis, which avoids cementoblastic differentiation in the follicle and cementum deposition or osteodentin next to the enamel ten cate 23 j oral pathol med. Adam17 is a poor prognostic indicator for patients with. Mutagenesis of the p53 tumorsuppressor gene represents the most common genetic alteration in human malignancies but has not yet been investigated in klatskin tumors. Klatskin tumors endoscopic diagnosis and therapy t. 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.
The disease was named after g erald klat skin, who in 1965 described 15 cases and found some characteristics for this type of cholangiocarcinoma. The klatskin tumor group had poorer rates of os and cancerspecific survival than the icca and hcc groups. Liver surgery for metachronous hepatic metastases with uterine body and uterine cervix origin a single center experience 15 decembrie 2016. During surgery they dicovered the liver was infected by 80%. Nov 06, 2019 ferrero a, lo tesoriere r, vigano l, caggiano l, sgotto e, capussotti l. 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. The 5year survival of gbc patients is reported to be between 0% and 10% in most reported series. Typically, these tumors are small, poorly differentiated, exhibit aggressive biologic behavior, and tend to obstruct the intrahepatic bile ducts. 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. Klatskin tumor article about klatskin tumor by the free. Nomograms predict survival outcome of klatskin tumors patients. 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. Tumor markers as a diagnostic key for hilar cholangiocarcinoma. Cancerous and normal liver tissues were obtained from 12 patients after surgical resection of klatskin tumors.
430 1479 418 1372 1607 505 1466 1549 347 779 1544 1242 20 1146 939 1058 1156 1314 1574 886 872 132 262 226 195 1187 843 326 1342 669 649 497 1202 682 363 878 987