Diagnosis and treatment of cholangiocarcinoma

Epidemiologic studies have demonstrated increasing mortality rates from intrahepatic cholangiocarcinoma during the past decades. Primary sclerosing cholangitis is the most important predisposing condition to the development of cholangiocarcinoma. Improvements in noninvasive diagnostic techniques have led to decreased use of invasive procedures. Magnetic resonance imaging (MRI) has the potential to depict parenchymal, ductal, and vascular tumor involvement. However, diagnosis can be difficult, and often ultrasonography, MRI, CT, and invasive cholangiography are complementary investigations. Genetic aberrations in brush cytology specimens should be explored further in prospective studies. Endoscopic ultrasonography, intraductal ultrasonography, and positron emission tomography are interesting techniques that are under evaluation. Radical surgery with negative histologic margins is the only curative option in cholangiocarcinoma. With more aggressive surgical approaches, including partial hepatectomy, 3-year survival rates of 35% to 50% can be achieved. Liver transplantation for unresectable cholangiocarcinoma was shown to be feasible in pilot studies of highly selected patients.     

Diagnosis and management of cholangiocarcinoma

Cholangiocarcinoma (CC) is a rare yet frequently fatal tumor that causes significant morbidity and mortality due to late presentation. Radiology is the mainstay of CC diagnosis; however, advances in understanding the pathogenesis of CC, including the role of oncogenes, inflammation-mediated genomic instability, and interleukin-6/STAT-3 signaling pathways, may allow development of new diagnostic and prognostic markers and targets for CC therapy. Although surgical resection is the standard of care for resectable CC, liver transplantation has shown excellent results in selected patients. The use of chemotherapy and radiotherapy are currently limited by marginal response rates, toxicity, and biliary complications. Locally ablative therapies in the form of transcatheter arterial chemoembolization and radioembolization are under investigation. Molecular therapies (eg, epidermal growth factor receptor, ErbB-2, and vascular endothelial growth factor receptor antagonists) and immunotherapy using diabodies are also under investigation for treatment of unresectable CC.     

Diagnosis of cholangiocarcinoma in primary sclerosing cholangitis

Introduction: Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the hepatobiliary system characterized by chronic inflammation, progressive fibrosis, stricture formation and destruction of extrahepatic and intrahepatic bile ducts.

Areas covered: The increased incidence of cholangiocarcinoma (CCA) in PSC has been well documented and can be explained by the continuous inflammation in the biliary tree leading to an enhanced dysplasia–carcinoma sequence. Although PSC patients may progress to liver cirrhosis; CCA most commonly occurs between the ages of 30 and 45 years when cirrhosis has not yet developed. Therefore, CCA in patients with PSC occurs earlier than in patients without PSC.

Expert commentary: Despite improvement in diagnostic methods and devices, the dilemma of diagnosing CCA in patients with PSC has not been solved yet and needs further investigation.     

Diagnosis,staging, and treatment of cholangiocarcinoma

Opinion statement Attention has to be focused on earlier detection of cholangiocarcinoma, especially in high-risk patients such as those with primary sclerosing cholangitis (PSC). Enhanced techniques for diagnosing this disease include imaging studies such as positron emission tomography (PET) scanning, and cytologic analysis including digital image analysis (DIA) and fluorescent in situ hybridization (FISH). Magnetic resonance imaging and endoscopic ultrasonography have also improved and simplified preoperative staging. Despite recent advances in the endoscopic management of cholangiocarcinoma, this neoplastic disease is still fatal in the majority of patients. Surgical resection or liver transplantation offers the only possibility for curing this disease in its early stages. However, most patients present with advanced disease that is not amenable to such therapy. For those patients presenting with unresectable carcinoma above the cystic duct without intrahepatic or extrahepatic metastases, orthotopic liver transplantation combined with preoperative irradiation and chemotherapy is available and demonstrates improved survival on the basis of a recent study conducted at the Mayo Clinic. In the future, chemopreventive strategies aimed at blocking the links between inflammation (eg, nitric oxide synthase [iNOS] and cylcooxygenase 2 [COX-2] inhibitors) and carcinogenesis may help prevent this often fatal disease in high-risk patients (eg, patients with PSC).     

Diagnosis and surgical treatment of primary hepatic cholangiocarcinoma

BACKGROUND/AIMS: To summarize the experience in the diagnosis and surgical treatment of primary hepatic cholangiocarcinoma (PHCC). METHODOLOGY: Forty-seven PHCC patients, who had undergone surgical treatment in our hospital from 1970 to 1999, were retrospectively analyzed with their clinicopathological features, surgical treatments and prognoses. RESULTS: 1) PHCC patients lacked typical clinical manifestation, and some had an accompanying history of virus B hepatitis (36.2%, 17/47) and liver cirrhosis (46.8%, 23/47), and elevation of AFP (25.5%, 12/47). 2) The detection rates of BUS, CT, MRI and liver scintigraphy were 95.7% (45/47), 93.6% (44/47), 100% (23/23) and 100% (4/4), respectively. The lesions detected by those methods needed to differentiate with hepatocellular carcinoma. 3) The resection rate was 76.6% (36/47), the postoperative overall 1-, 3- and 5-year survival rates were 54.1% (24/47), 34.0% (16/47), and 29.8% (14/47) in all patients, respectively, including the 1-, 3- and 5-year survival rates were 83.3% (30/36), 50.0% (18/36), and 38.9% (14/36) in hepatectomy cases, respectively. 4) All of the four patients who survived more than 5 years, including small liver cancer (< or = 3 cm) three cases and encapsulated large nodular lesion one case, received curable resection and postoperative adjuvant therapies. CONCLUSIONS: PHCC patients can obtain a better result by early detection and curative resection with adjuvant therapies.     

Diagnosis and initial management of cholangiocarcinoma with obstructive jaundice

Cholangiocarcinoma is the second most common primary hepatic cancer. Despite advances in diagnostic techniques during the past decade, cholangiocarcinoma is usually encountered at an advanced stage. In this review, we describe the classification, diagnosis, and initial management of cholangiocarcinoma with obstructive jaundice.     

胆管癌诊断与治疗——外科专家共识

<正>胆管癌(cholangiocarcinoma)统指胆管系统衬覆上皮发生的恶性肿瘤,按所发生的部位可分为肝内胆管癌(intrahepatic cholangiocarcinoma,ICC)和肝外胆管癌(extrahepatic cholangiocarcinoma,ECC)两大类。ICC起源于肝内胆管及其分支至小叶间细胆管树的任何部位的衬覆上皮;ECC又以胆囊管与肝总管汇合点为界分为肝门部胆管癌和远端胆管癌。近年来胆管癌的发病率逐年升高,对于胆管癌的诊断及治疗,国外早在     

螺旋CT 和MRI 对肝内周围型胆管癌诊断价值分析

目的探讨螺旋计算机断层成像（CT）和磁共振成像（MRI）技术对肝内周围型胆管癌（IHPCC）的诊断价值。方法经病理学检查证实的80例IHPCC患者,均行螺旋CT和MRI平扫、增强和延迟扫描检查,比较影像学检查诊断的正确率。结果影像学上病灶为1.3 cm×2.0 cm~7.3 cm×9.0 cm （平均4.6 cm×6.4 cm）;位于肝左叶58例,肝右叶22例;呈肿块型46例（57.50%）、浸润狭窄型17例（21.25%）、腔内生长型10例（12.50%）、肿块不明确7例（8.75%）;CT平扫病灶呈略低密度,增强扫描时病灶分别呈轻中度的边缘强化50例、中央轻度条状或片状强化12例和无强化18例,延迟扫描时呈不均匀性片状或分隔状强化47例、均匀强化5例和无强化28例;MRI平扫较CT显示病灶更为清晰,延迟后73例病灶逐渐强化;MRI诊断正确率为91.3%,CT诊断正确率为80.0%,两者比较差异无统计学意义（P>0.05）。结论增强延迟扫描可提高螺旋CT和MRI诊断IHPCC的准确性,诊断IHPCC时可优先考虑行MRI检查。     

中晚期肝门部胆管癌诊治15例

目的:探讨中晚期肝门部胆管癌的外科治疗方式选择,提高手术切除率和疗效.方法:对2001-10/2004-12收治的15例中晚期肝门部胆管癌的外科治疗方式和随访结果进行回顾性分析,其中中期7例(BismuthIIIa型4例,IIIb型3例),晚期(IV型)8例.结果:手术切除10例,非手术胆道支架内引流5例(IV型5例)随访14例(14/15,93.3%),手术切除组随访10例,中位生存期14.2mo(16d-32.3mo);非手术胆道支架内引流组随访4例,中位生存期3.8mo(1.3-7.2mo);两组中位生存期差异显著(t=2.802,P<0.05).结论:对于中晚期肝门部胆管癌,联合肝切除的姑息性肝门部胆管癌切除可显著延长患者生存期、提高生活质量,必要时辅以“架桥式”肝管空肠内引流对提高手术切除率、降低术后胆漏发生率具有重要作用.     

MSCT多期增强扫描诊断肝门区胆管癌15例

目的:评价MSCT多期动态增强扫描对肝门胆管癌的诊断价值.方法:回顾性分析15例经病理确诊的肝门区胆管癌的临床资料及MSCT图像特征,所有病例均行平扫及四期动态增强扫描(即早动脉期、晚动脉期、门脉期及延迟期).结果:15例肝门胆管癌按CT表现形式分为肿块型、结节型及管壁浸润型,所有病灶均表现为等密度或略低密度.7例肿块型动脉期肿块边缘呈轻度不均匀强化,晚动脉期、门静脉期及延迟期病灶进一步强化:5例结节型多表现为肝门部<2 cm的结节,动脉期主要为边缘环状强化,门静脉期及延迟期持续明显强化,且向中心部充填:3例管壁浸润型表现为肝门部胆管壁局限性不规则增厚>2 mm,增厚的胆管壁四期扫描均呈明显环状强化,其中2例表现为延迟强化.     

Diagnosis and treatment of extrahepatic cholangiocarcinoma: results in a series of 124 patients

AIM: The aim of the study was to evaluate the diagnostic methods and therapeutical results in patients with extrahepatic cholangiocarcinoma admitted between 2000 and 2003 and to assess the efficacy of various imaging methods. MATERIAL AND METHOD: We included 124 consecutive patients with suspected cholangiocarcinoma who underwent endoscopic retrograde cholangiopancreatography (ERCP) between 2000 and 2003. Every patient underwent an ultrasound (US) examination before ERCP. ERCP was considered as the "gold standard" for diagnosis. The therapy applied consisted of endoscopic stenting, US guided biliary drainage or surgery. The sensitivity, specificity and accuracy were calculated for every tumour localisation and also globally for all tumours. RESULTS: The sensitivity, specificity and accuracy of US were 85.9 %, 76.9 %, and 84.4 % for hilar localization, 59.1 %, 50 % and 57.1 % for the mid bile duct (CBD) and 33.3 %, 42.8 % and 36.8 % for the distal CBD tumours. The global performances for US in diagnosing extrahepatic cholangiocarcinoma were 73.5 %, 61.5 % and 70.9 %. In 73 cases (74.5%) an endoscopic stent was placed. In 11 cases (11.2%) we performed an US guided biliary drainage and in 14 cases (14.2%) surgery was recommended. CONCLUSIONS: Ultrasonography proved to be a reliable method for the diagnosis of cholangiocarcinoma in spite of its low accuracy for distal localization. The combination of the two investigations (US and ERCP) was very efficient in the management of these patients. Endoscopic stenting for hilar localization is a good therapeutical option.     

Evaluation and endoscopic palliation of cholangiocarcinoma. Management of cholangiocarcinoma

Patients with a cholangiocarcinoma usually develop symptoms of biliary obstruction which dominate the course of their illness. At presentation, the diagnosis may be difficult to establish due to the desmoplastic nature of this tumor. Location and extent of the tumor, however, are more effectively evaluated by current radiologic techniques. Following these staging procedures, most patients are found to be unsuitable for curative resection. Therefore, establishing effective biliary drainage to relieve symptoms of obstruction becomes the most critical therapeutic goal in these patients. In the past, surgical biliary bypass procedures were advocated, but were associated with high perioperative morbidity and mortality rates. Endoscopic biliary stenting produces quick and effective relief of symptoms from biliary obstruction due to cholangiocarcinoma. Initially, successful biliary drainage is possible in most patients. However, subsequent stent occlusion or spread of tumor into nonstentable intrahepatic ducts leads to recurrent jaundice. Local therapies including (192)Ir brachytherapy and photodynamic therapy may prevent or postpone these problems.     

Surgical management of cholangiocarcinoma

Biliary tract cancer affects approximately 7500 Americans each year. Tumors arising from the gallbladder are the most common; those of bile duct origin, or cholangiocarcinoma, are less frequently encountered, constituting approximately 2% of all reported cancers. Although cholangiocarcinoma can arise anywhere within the biliary tree, tumors involving the biliary confluence (i.e., hilar cholangiocarcinoma) represent the majority, accounting for 40 to 60% of all cases. Twenty to 30% of cholangiocarcinomas originate in the lower bile duct, and approximately 10% arise within the intrahepatic biliary tree and will present as an intrahepatic mass. Complete resection remains the most effective and only potentially curative therapy for cholangiocarcinoma. For all patients with intrahepatic cholangiocarcinoma and nearly all patients with hilar tumors, complete resection requires a major partial hepatectomy. Distal cholangiocarcinomas, on the other hand, are treated like all periampullary malignancies and typically require pancreaticoduodenectomy. Most patients with cholangiocarcinoma present with advanced disease that is not amenable to surgical treatment, and even with a complete resection, recurrence rates are high. Adjuvant therapy (chemotherapy and radiation therapy) has not been shown clearly to reduce recurrence risk.     

Cutaneous metastasis of cholangiocarcinoma

AIM:To investigate the clinical characteristics and prognostic factors of cutaneous metastasis of cholangiocarcinoma by a retrospective analysis of published cases.METHODS:An extensive search was conducted in the English literature within the Pub Med database using the following keywords:cutaneous metastasis or skin metastasis and cholangiocarcinoma or bile duct.The data of 30 patients from 21 articles from 1978 to 2014 were analyzed.Patient data retrieved from the articles included the following:age,gender,time cutaneous metastasis occurred,number of cutaneous metastases throughout life,sites of initial cutaneous metastasis,anatomic site,pathology and differentiation of cholangiocarcinoma,and immunohistochemical results of the cutaneous metastasis.The assessment of overall survival after cutaneous metastasis(OSCM) was the primary endpoint.RESULTS:The median age at diagnosis of cutaneous metastasis of cholangiocarcinoma was 60.0 years(range:35-77).This metastasis showed a predilection towards males,with a male to female ratio of 3.29.In 8 cases(27.6%),skin metastasis was the first sign of cholangiocarcinoma.Additionally,18 cases(60.0%) manifested single cutaneous metastasis,while 12 cases(40.0%) demonstrated multiple skin metastases.In 50.0% of patients,the metastasis occurred in the drainage region,while 50.0% of patients had distant cutaneous metastases.The scalp was the most frequently involved region of distant skin metastasis,occurring in 36.7% of patients.The median OSCM of cholangiocarcinoma was 4.0 mo.Patient age and cutaneous metastatic sites showed no significant relation with OSCM,while male gender and single metastasis of the skin were associated with a poorer OSCM(hazard ratio:0.168;P = 0.005,and hazard ratio:0.296;P = 0.011,respectively).CONCLUSION:The prognosis of cutaneous metastasis of cholangiocarcinoma is dismal.Both male gender and single skin metastasis are associated with a poorer OSCM.