Klatskin tumor diagnosed concurrently with IgG4 related sclerosing cholangitis: A case report.

RATIONALE: IgG4-related disease (IgG4-RD) is a systemic disease that can involve various organs and is characterized by the infiltrations of IgG4-positive plasma cells and lymphocytes, fibrosis, and elevated serum IgG4 levels. IgG4-related sclerosing cholangitis (IgG4-RSC) is a subtype of IgG4-RD. No certain relationship between IgG4-RSC and cholangiocarcinoma has been established as yet, and there have been few reports of the simultaneous diagnosis of IgG4-RSC and cholangiocarcinoma. PATIENT CONCERNS: A 76-year-old male visited our gastroenterology department due to the recent occurrence of pruritus and jaundice. DIAGNOSIS: Computed tomography (CT) scan showed ductal wall swelling and enhancement from both intrahepatic duct confluence to the common bile duct, upper biliary dilatation, and accompanying autoimmune pancreatitis (a sub type of IgG4-RD). Biopsy of the distal common bile duct by endoscopic retrograde cholangiopancreatography (ERCP) resulted in a diagnosis of IgG4-RSC. Subsequently, adenocarcinoma was identified by repeated cytology of bile juice. Finally, Klatskin tumor type IIIA and IgG4-RSC were concurrently diagnosed. INTERVENTIONS: IgG4-RSC was treated with steroid and Klatskin tumors by gemcitabine + cisplatin chemotherapy. OUTCOMES: The jaundice had improved and CT showed substantial improvement of the intrahepatic duct dilatation. LESSONS: IgG4-RSC and cholangiocarcinoma are easily confused, but their treatments are quite different, and thus, care must be taken during diagnosis. Furthermore, these 2 diseases may co-exist. Therefore, even if IgG4-RSC is diagnosed first, the possibility of accompanying cholangiocarcinoma should be thoroughly investigated.

Surgical Strategies for the Treatment of Bismuth Type I and II Hilar Cholangiocarcinoma: Bile Duct Resection with or Without Hepatectomy?

BACKGROUND: The role of hepatic resection in the treatment of type I and II hilar cholangiocarcinoma (HCCA) remains controversial. In the present study, we aimed to identify whether hepatic resection was necessary for type I and II HCCA. METHODS: A total of 23 patients classified as type I and II HCCA undergoing surgical resection were included in this study. The patients were divided into two groups: bile duct resection (BDR) group (n = 15) and hepatic resection (HR) group (n = 8). Systematic review and meta-analysis were performed to compare the R0 resection and long-term survival between BDR and HR for Bismuth type I and II HCCA. A total of 7 studies with 260 cases were included in this meta-analysis. RESULTS: In our cohort, the R0 resection rate was 73.3% in BDR group and 87.5% in HR group. The HR group had a higher number of postoperative complications than the BDR group (P = 0.002). There was no difference in long-term survival (P = 0.544) and recurrence (P = 0.846) between BDR and HR in Bismuth type I and II HCCA. The meta-analysis showed that HR was associated with better R0 resection rate (RR 4.45, 95% CI 2.34-8.48) and overall survival (HR 2.15, 95% CI 1.34-3.44) compared with BDR group. There was no publication bias and undue influence of any single study. CONCLUSIONS: The meta-analysis showed that HR was associated with better R0 resection rate and overall survival compared with BDR for type I and II HCCA patients. More aggressive surgical strategies should be increasingly considered for the treatment of type I and II HCCA patients.

Prognostic impact of the Bismuth-Corlette classification: Higher rates of local unresectability in stage IIIb hilar cholangiocarcinoma.

BACKGROUND: The Bismuth-Corlette (BC) classification is used to categorize hilar cholangiocarcinoma by proximal extension along the biliary tree. As the right hepatic artery crosses just behind the left bile duct, we hypothesized that BC IIIb tumors would have a higher likelihood of local unresectability due to involvement of the contralateral artery. METHODS: A retrospective review of a prospectively maintained database identified patients with hilar cholangiocarcinoma taken to the operating room for intended curative resection between April 2008 and September 2016. Cases were assigned BC stages based on preoperative imaging. RESULTS: Sixty-eight patients were included in the study. All underwent staging laparoscopy after which 16 cases were aborted for metastatic disease. Of the remaining 52 cases, 14 cases were explored and aborted for locally advanced disease. Thirty-eight underwent attempt at curative resection. After excluding cases aborted for metastatic disease, the chance of proceeding with resection was 55.6% for BC IIIb staged lesions compared to 80.0% of BC IIIa lesions and to 82.4% for BC I-IIIa staged lesions (P < 0.05). About 44.4% of BC IIIb lesions were aborted for locally advanced disease versus 17.6% of remaining BC stages. CONCLUSIONS: When hilar cholangiocarcinoma is preoperatively staged as BC IIIb, surgeons should anticipate higher rates of locally unresectable disease, likely involving the right hepatic artery.

Cystic duct cancer: Should it be deemed as a type of gallbladder cancer?

BACKGROUND: According to the latest American Joint Committee on Cancer and Union for International Cancer Control manuals, cystic duct cancer (CC) is categorized as a type of gallbladder cancer (GC), which has the worst prognosis among all types of biliary cancers. We hypothesized that this categorization could be verified by using taxonomic methods. AIM: To investigate the categorization of CC based on population-level data. METHODS: Cases of biliary cancers were identified from the Surveillance, Epidemiology, and End Results 18 registries database. Together with routinely used statistical methods, three taxonomic methods, including Fisher's discriminant, binary logistics and artificial neuron network (ANN) models, were used to clarify the categorizing problem of CC. RESULTS: The T staging system of perihilar cholangiocarcinoma [a type of extrahepatic cholangiocarcinoma (EC)] better discriminated CC prognosis than that of GC. After adjusting other covariates, the hazard ratio of CC tended to be closer to that of EC, although not reaching statistical significance. To differentiate EC from GC, three taxonomic models were built and all showed good accuracies. The ANN model had an area under the receiver operating characteristic curve of 0.902. Using the three models, the majority (75.0%-77.8%) of CC cases were categorized as EC. CONCLUSION: Our study suggested that CC should be categorized as a type of EC, not GC. Aggressive surgical attitude might be considered in CC cases, to see whether long-term prognosis could be immensely improved like the situation in EC.

[Minor liver resection for hilar cholangiocarcinoma of Bismuth-Corlette type â ¢ and â £].

Objective: To explore the clinical effect and safety of minor liver resection for hilar cholangiocarcinoma (HC) of Bismuth-Corlette type Ⅲ and Ⅳ. Methods: From May 2007 to May 2017, the clinical data of 108 patients with Bismuth-Corlette type Ⅲ and Ⅳ HC underwent hepatectomy were collected and analyzed retrospectively.There were 56 males and 52 females, aged (57.2±5.3) years (ranged 48-76 years) .Among the 108 cases, there were 51 cases of type Ⅲa, 40 cases of type Ⅲb and 17 cases of type Ⅳ. Small-scale hepatectomy (≤3 hepatectomy) was performed in 70 cases, including 8 cases of 4b segment resection, 28 cases of 4b segment+5 segment resection, and 34 cases of partial 4 segment+partial 7 segment+partial 1 segment resection. Large-scale hepatectomy was performed in 38 cases (>3 segments) , of which 30 cases were treated with 2 segments+3 segments+4 segments+1 segment, and 8 cases were treated with 5 segments+7 segments+8 segments+1 segment. t' test was used to analyze the data which did not conform to the normal distribution, and χ(2) test was used to calculate the incidence of postoperative complications and the 1, 3, and 5-year cumulative overall survival rate. Results: (1) The operation time of minor liver resection group ((180±25)minutes) was shorter than that of major liver resection group ((210±35)minutes) (t'=4.676, P<0.05) , the amount of blooding operation time of minor liver resection group ((310±80)ml) was less than that of major liver resection group ((500±110)ml)in the operation (t'=9.385, P<0.05) , and the difference was statistically significant. (2) The incidence of complications was lower in minor liver resection group and major liver resection group, and the difference was statistically significant (χ(2)=5.230, P<0.05) . (3) The actual 1-, 3- and 5-year survival rates were 87.1%, 58.4%, 30.0% and 84.2%, 57.9%,31.6%, respectively. There were no significant differences in survival rates in two groups in 1-, 3- and 5-year survival rates (χ(2)=0.177, P=0.674; χ(2)=0.005, P=0.946; χ(2)=0.029, P=0.865) . Conclusions: Compared to patients with major liver resection, Minor liver resection for selected patients with HC of Bismuth-Corlette Ⅲ and Ⅳaccording to our criteria achieved better long-term outcomes. Chen's biliojejunostomy is a simple, effective and safe method, which can be widely used when there are multiple biliary intestinal anastomosese.

Long-term outcome and prognostic factors of intrahepatic cholangiocarcinoma involving the hepatic hilus versus hilar cholangiocarcinoma after curative-intent resection: Should they be recognized as perihilar cholangiocarcinoma or differentiated?

BACKGROUND: Perihilar cholangiocarcinoma is defined as tumors arising predominantly at or near the biliary confluence, potentially consisting of two types: hilar cholangiocarcinoma (HC) and intrahepatic cholangiocarcinoma involving the hepatic hilum (hICC). However, whether hICC and HC should be strictly distinguished or combined remains highly controversial. We aimed to compare the clinicopathological characteristics, prognostic factors and long-term outcome of hICC versus HC after curative-intent resection. METHODS: Between January 1998 and June 2015, a total of 325 patients with hICC (n = 146) and HC (n = 179) who underwent curative-intent resection were enrolled. The medical records of these patients were retrospectively reviewed. RESULTS: Portal vein invasion, larger tumors, and later T stage were significantly more common in hICC group. A total of 110 (75.3%) hICC patients and 119 (66.5%) HC patients experienced tumor recurrences, respectively. The median recurrence-free survival (RFS) and overall survival (OS) of hICC patients were significantly worse than those of HC patients (median RFS, 14.3 versus 22.7 months, P = 0.014; median OS, 21.7 versus 30.6 months, P = 0.032). Multivariate analysis revealed tumor size, satellite nodules, surgical margin, and histological grade as independent factors for OS in hICC patients. On the other hand, the presence of liver parenchyma invasion, portal invasion, lymphovascular invasion, later N stage, and positive surgical margin were associated with shorter OS in HC patients. CONCLUSIONS: hICC showed distinct clinicopathological features, more aggressive biological behaviors, different prognostic factors, and worse prognosis in comparison with HC. Therefore, making a strict distinction between hICC and HC is necessary.

Proposal for a new classification for perihilar cholangiocarcinoma based on tumour depth.

BACKGROUND: The T system for distal cholangiocarcinoma has been revised from a layer-based to a depth-based approach in the current American Joint Committee on Cancer (AJCC) classification. In perihilar cholangiocarcinoma, tumour depth in the staging scheme has not yet been addressed. The aim of this study was to propose a new T system using measured tumour depth in perihilar cholangiocarcinoma. METHODS: Patients who underwent hepatectomy for perihilar cholangiocarcinoma between 2001 and 2014 were reviewed retrospectively. The vertical distance between the top of the tumour and deepest invasive cells was measured as invasive tumour thickness (ITT) by two independent pathologists. Log rank statistics were used to determine cut-off points, and the concordance (C) index was used to assess survival discrimination of each T system. RESULTS: ITT was measurable in all 440 patients, with a median value of 6·0 (range 0-45) mm. The median difference in ITT between observers was 0·6 (range 0-20) mm. Cut-off points for prognosis were 1, 5 and 8 mm. Five-year survival decreased with increasing ITT (P < 0·001): 67 per cent for ITT less than 1 mm (25 patients), 54·9 per cent for ITT 1 mm and over to less than 5 mm (138 patients), 43·4 per cent for ITT 5 mm and over to less than 8 mm (118 patients), and 32·2 per cent for ITT 8 mm and over (159 patients). The C-index of this classification was comparable to that of the current AJCC T classification (0·598 versus 0·589). CONCLUSION: ITT is a reliable approach for making a depth assessment in perihilar cholangiocarcinoma. A four-tier ITT classification with cut-off points of 1, 5 and 8 mm is an adequate alternative to the current layer-based T classification.

Surgical resection for Bismuth type IV perihilar cholangiocarcinoma.

BACKGROUND: Bismuth type IV perihilar cholangiocarcinoma has traditionally been categorized as unresectable disease. The aim of this study was to review experience with a resection-based strategy in patients who have type IV perihilar cholangiocarcinoma. METHODS: Medical records of consecutive patients with a diagnosis of type IV perihilar cholangiocarcinoma between 2006 and 2015 were reviewed retrospectively. Primary outcomes assessed were surgical results and long-term survival. RESULTS: Of the 332 patients with type IV tumour, 216 (65·1 per cent) underwent resection. Left hepatic trisectionectomy was the most common procedure (112 patients). Combined vascular resection was performed in 131 patients. Median duration of operation was 607 (range 356-1045) min, and blood loss was 1357 (209-10 349) ml. Complications of Clavien-Dindo grade III or more developed in 90 patients (41·7 per cent) and four (1·9 per cent) died from complications within 90 days. Survival rates were better for the 216 patients whose tumours were resected than for the 116 patients with unresected tumours (32·8 versus 1·5 per cent at 5 years; P < 0·001). Patients with pN0 M0 disease after resection had a favourable 5-year survival rate of 53 per cent. Percutaneous transhepatic biliary drainage, blood transfusion, lymph node metastasis and distant metastasis were identified as independent negative prognostic factors for survival. CONCLUSION: Although resection for type IV tumour is technically demanding with high morbidity, it can be performed with low mortality and offers better survival probability in selected patients.

Intrahepatic, Perihilar and Distal Cholangiocarcinoma: Management and Outcomes

Abstract: Introduction and aims. Cholangiocarcinomas are a heterogeneous group of tumors that can be classified into three clinically distinct types of cancers, intrahepatic, perihilar and distal cholangiocarcinoma. The inconsistent use of nomenclature for these cancers has obscured a true knowledge of the epidemiology, natural history and response to therapy of these cancers. Our aims were to define demographic characteristics, management and outcomes of these three distinct cancer types. Materials and methods. A retrospective study of patients enrolled in an institutional cancer registry from 1992 to 2010. Median survival was compared between different treatment modalities over three time periods for the three types of cholangiocarcinoma at different stages of the disease using Kaplan Meyer analysis. Results. 242 patients were identified. All cases were reviewed and classified into intrahepatic (90 patients), distal (48 patients) or perihilar (104 patients) cholangiocarcinomas. These cancers differed in median age of onset, gender distribution, median survival and stage. 13.8% of patients presented with stage I, 5.8% with stage II, 9.6% with stage III, 28% with stage IV, with 41.8% having unknown stage. The overall median survival was 15.8 months, and was 23, 25, 14, and 4.5 months for stages I, II, III, and IV respectively. Surgery improved survival in both early and advanced stages. Multimodality therapies further improved outcomes, particularly for perihilar cholangiocarcinoma. Conclusion. Perihilar, distal and intrahepatic cholangiocarcinoma vary in their presentation, natural history and therapeutic approach to management. A consistently applied classification is essential for meaningful interpretation of studies of these cancers.

Intrahepatic, perihilar and distal cholangiocarcinoma: Management and outcomes.

 Introduction and aims. Cholangiocarcinomas are a heterogeneous group of tumors that can be classified into three clinically distinct types of cancers, intrahepatic, perihilar and distal cholangiocarcinoma. The inconsistent use of nomenclature for these cancers has obscured a true knowledge of the epidemiology, natural history and response to therapy of these cancers. Our aims were to define demographic characteristics, management and outcomes of these three distinct cancer types. MATERIALS AND METHODS: A retrospective study of patients enrolled in an institutional cancer registry from 1992 to 2010. Median survival was compared between different treatment modalities over three time periods for the three types of cholangiocarcinoma at different stages of the disease using Kaplan Meyer analysis. RESULTS: 242 patients were identified. All cases were reviewed and classified into intrahepatic (90 patients), distal (48 patients) or perihilar (104 patients) cholangiocarcinomas. These cancers differed in median age of onset, gender distribution, median survival and stage. 13.8% of patients presented with stage I, 5.8% with stage II, 9.6% with stage III, 28% with stage IV, with 41.8% having unknown stage. The overall median survival was 15.8 months, and was 23, 25, 14, and 4.5 months for stages I, II, III, and IV respectively. Surgery improved survival in both early and advanced stages. Multimodality therapies further improved outcomes, particularly for perihilar cholangiocarcinoma. CONCLUSION: Perihilar, distal and intrahepatic cholangiocarcinoma vary in their presentation, natural history and therapeutic approach to management. A consistently applied classification is essential for meaningful interpretation of studies of these cancers.

[View on the problem of Klatskin tumor].

Klatskin tumor arises from the common hepatic duct and its bifurcation the bile ducts and is the most common primary malignancy of the biliary tree. the location of the tumor and its close relationship with vascular structures at the hepatic hilum have resulted in a low resectability and high morbidity and mortality. Improvement of instrumental diagnostics and operative techniques allows to perform extended resection and complex interventions on the liver, bile ducts and vascular structures at the hepatic hilum. The role of chemoratiotherapy and photodynamic therapy is not fully understood. thus, questions of treatment and prognosis of the disease are remain relevant and require further study.

A modified Jarnagin-Blumgart classification better predicts survival for resectable hilar cholangiocarcinoma.

BACKGROUND: Prediction of postoperative survival for hilar cholangiocarcinoma (HCCA) remains difficult although there have been a variety of clinical classification and staging systems. This study was designed to validate and compare some of the major HCCA staging systems in use today. In addition, we sought to build up a new staging system modified from Jarnagin-Blumgart (J-B) classification for HCCA, to predict survival better. METHODS: A total of 154 consecutive cases of HCCA including 95 surgical patients between 2005 and 2014 were enrolled in this study. The clinical and pathological data were recorded retrospectively and three commonly used classification methods: Bismuth-Corlette (B-C) classification, TNM staging, and J-B classification were performed to analyze the correlations with resectability and survival. Chi-square test, Kaplan-Meier analysis, and kappa statistics were used to compare and confirm the relationships between the variables and survival. RESULTS: For all 154 patients, the resection rate of J-B T1 was 68.6% (48/70), higher than that of J-B T2 (44.8%, P = 0.007). J-B T2 also showed a higher resectability than J-B T3 (19.2%, P = 0.025). There was no significant difference in resectability within the groups B-C type and TNM stages. We set up a new staging system based on J-B classification, tumor differentiation, distant metastasis (N2 or M1 of TNM stage), and resection integrality. The total survival predictive accuracy was 69.5% (kappa = 0.547), higher than that of TNM staging and J-B classification. CONCLUSIONS: J-B classification was more useful than B-C classification, while its value for predicting survival did not exceed TNM staging system. The new staging system, based on J-B classification, provides a better method to stratify HCCA patients during the operation.

Secular trends in the incidence of cholangiocarcinoma in the USA and the impact of misclassification.

BACKGROUND AND AIMS: It has been reported that the incidence of intrahepatic cholangiocarcinoma (ICC) has increased in the USA, while extrahepatic cholangiocarcinoma (ECC) has decreased or remained stable. However, neither the recent trends nor the effects of the misclassification of Klatskin tumors are known. METHODS: Using the Surveillance, Epidemiology, and End Results program databases, we calculated the average annual age-adjusted incidence rates (AA-IRs) of ICC and ECC in 4-year time periods (1992-1995, 1996-1999, 2000-2003, 2004-2007). These AA-IRs were calculated with misclassified as well as correctly classified Klatskin tumors. AA-IRs were also calculated based on age, sex, and race. Multivariable Poisson regression models were used to evaluate the secular trends of ICC and ECC. RESULTS: The AA-IR of ICC was 0.92 in 1992-1995 and 0.93 in 2004-2007, while the AA-IR of ECC increased from 0.70 in 1992-1995 to 0.95 in 2004-2007. There was no significant trend in AA-IR of ICC (p = 0.07), while there was a significant increase in ECC across the 4-year time periods (p < 0.001). Klatskin tumors comprised 6.7% of CCs with approximately 90 and 45% misclassified as ICC during 1992-2000 and 2001-2007, respectively. Adjusted Poisson models showed no significant differences in the temporal trend of ICC or ECC due to misclassification of Klatskin tumors. CONCLUSIONS: The incidence of ICC has remained stable between 1992 and 2007 with only slight fluctuations, while the incidence of ECC has been increasing. Misclassification of Klatskin tumors does not appear to play a significant role in the trends of CCs.

[Pathological anatomical characteristics of Klatskin tumors. Classification, current molecular biological aspects, prognosis factors]. / Anatomisch-pathologische Besonderheiten der Klatskin-Tumoren: Klassifikation, aktuelle molekular-biologische Aspekte, Prognosefaktoren.

Klatskin tumors are a distinct subgroup of cholangiocarcinomas which are a surgical challenge due to their special localization. Different localizations do not show great differences concerning histomorphology and precursor lesions. With respect to molecular alterations there are only small differences. Accurate clinical and histomorphological diagnosis is important for therapy and especially the prediction of prognosis as well as standardized processing of the resection specimen if the carcinoma is resectable. Additionally, accurate lymph node dissection is necessary. Concerning molecular markers further investigations are needed to develop individualized therapy regimes.

Rising trends in cholangiocarcinoma: is the ICD classification system misleading us?

BACKGROUND & AIMS: Cholangiocarcinomas (CC) can be sub-divided into intrahepatic (IHCC) or extrahepatic (EHCC). Hilar or 'Klatskin' tumours are anatomically extrahepatic. Most international studies, also from the UK, report increasing IHCC and decreasing EHCC incidence. The second edition of the International Classification of Diseases for Oncology (ICD-O-2) assigned 'Klatskin' tumours a unique histology code (8162/3), but this was cross-referenced to the topography code for intrahepatic (IHBD) rather than extrahepatic bile duct tumours (EHBD). Under the third ICD-O edition, 'Klatskin' tumours are cross-referenced to either IHBD or EHBD. New editions of the ICD-O classification are adopted at different time points by different countries. We investigated the impact of changing ICD-O classifications and the potential misclassification of hilar/'Klatskin' tumours on bile duct tumour and CC incidence rates in England and Wales and the US. We also examined whether coding practices by cancer registries in England and Wales could be influencing these rates. METHODS: We analysed age-standardised incidence rates (ASIR) in England and Wales for IHBD and EHBD tumours between 1990 and 2008, then transferred all 'Klatskin' tumours from IHBD to EHBD and reanalysed rates from 1995, when ICD-O-2 was introduced in the UK. We also compared trends in IHBD, EHBD, and 'Klatskin' tumours in England and Wales with those in the USSEER (Surveillance, Epidemiology and End Results) database. Coding practice at Cancer registry level in England and Wales was investigated via a questionnaire completed by all national cancer registries. RESULTS: In England and Wales, 1990-2008, ASIR of IHBD cancers rose (0.43-1.84/100,000 population in males; 0.27-1.51 in females) but fell for EHBD (0.78-0.51/100,000 population in males; 0.62-0.39 in females). After transferring all 'Klatskin' tumours from IHBD to EHBD, there remained a marked increase in ASIR of IHBD cancers and a decrease in ASIR for EHBD, as only 1% of CC were reportedly 'Klatskin'. The US SEER data showed that ASIR for IHBD gradually rose from 0.59/100,000 population in 1990 to 0.91 in 2001, then sharply fell before plateauing at 0.60 by 2007. ASIR for EHBD remained relatively stable at around 0.80/100,000 population until 2001, then began increasing, to 0.97 by 2007. Annually, between 1995 and 2008, the vast majority of 'Klatskin' tumours in England and Wales were coded as IHBD. This was also the case in the SEER data until 2001, when the situation was reversed and subsequently most 'Klatskin' tumours were coded as EHBD. US trends coincide with a switch from ICD-O2 to ICD-O-3 in 2001. In the UK, the switch to ICD-O-3 only occurred in 2008. On questioning, cancer registries in England and Wales stated they would not code a CC described as 'hilar' with the designated 'Klatskin' histology code. If the tumour site is unspecified, most registries classify CC as intrahepatic. CONCLUSIONS: Changes in ICD-classification may be influencing observed changes in IHBD and EHBD incidence rates. Coding misclassification is likely to have been skewing CC registration to an intrahepatic site, thereby contributing to the previously reported rise in intrahepatic tumours.

[Classification and staging systems for hilar cholangio-carcinoma (Klatskin tumors): clinical application and practical relevance]. / Sisteme de clasificare si stadializare pentru colangio- carcinomul hilar (tumora Klatskin): aplicatie clinica si relevanta practica.

Hilar cholangiocarcinomas or Klatskin tumors have been classified in 1975 by French surgeons Henri Bismuth and Marvin B. Corlette and this remains largely used in clinical practice. The authors present the TNM classification and the changes introduced by the sixth and seventh edition of Union for International Cancer Control regarding the tumors of the proximal bile duct and describe Blumgart classification for tumors of this site. The usefulness of these systems is assessed considering the last six years experience of the service.

Cholangiocarcinoma--current treatment options.

The treatment for cholangiocarcinoma (CCA) remains a challenge because of the aggressive nature of the disease and the absence of effective treatments besides surgical resection (HR) and liver transplantation (LT). In intrahepatic CCA, HR remains the treatment of choice whereas with concomitant liver disease such as cirrhosis or primary sclerosing cholangitis (PSC), LT is the only option. Hilar CCA or Klatskin tumours have in recent decades been managed with a more aggressive surgical approach to achieve R0 resection. This approach usually involves preoperative portal embolisation, followed by liver resection ­ sometimes extensive and even with portal vein resection. The recent protocols that combine preoperative neoadjuvant chemoirridation and LT show promising results that need to be confirmed. The development of diagnostic modalities (tumour markers, cytology and radiology) are of the utmost importance to identify these patients at an early stage to preserve radical surgery possible. Cholangiocarcinoma (CCA) is a malignant disease of the epithelial cells in the intra- and extrahepatic bile ducts. While still a rare malignant disease, CCA is the second most common primary malignancy of the liver. The incidence is increasing; especially the incidence of intrahepatic CCA (1). The treatment of CCA is challenging as it is usually difficult to diagnose when radical surgical treatment, resection (HR) or liver transplantation (LT) is possible. The lack of effective medical treatment makes a radical surgical resection or hepatectomy the only therapeutic option. Most of the CCAs are unresectable at presentation and the prognosis for these patients is dismal.

Klatskin tumors and the accuracy of the Bismuth-Corlette classification.

The Bismuth-Corlette (BC) classification is the current preoperative standard to assess hilar cholangiocarcinomas (HC). The aim of this study is to evaluate the accuracy, sensitivity, and prognostic value of the BC classification. Data of patients undergoing resection for HC were analyzed. Endoscopic retrograde cholangiography and standard computed tomography were undertaken in all cases. Additional 3D-CT-reconstructions, magnetic resonance imaging, and percutaneous transhepatic cholangiography were obtained in selected patients. A systematic review and meta-analysis of the literature was performed. Ninety patients underwent resection of the hilar bile duct confluence, with right or left hemihepatectomy in 68 instances. The overall accuracy of the BC classification was 48 per cent. Rates of BC under- and over-estimation were 29 per cent and 23 per cent, respectively. The addition of MRI, 3D-CT-reconstructions, or percutaneous transhepatic cholangiography improved the accuracy to 49 per cent (P = 1.0), 53 per cent (P = 0.074), and 64 per cent (P < 0.001), respectively. Lowest sensitivity rates were for BC Type IIIA/IIIB tumors. Meta-analysis of published BC data corresponding to 540 patients did not reach significance. The BC classification has low accuracy and no prognostic value in cases of HC undergoing resection.

Tumor de Klatskin / Klatskin tumor

Epidemiología, factores de riesgo, clasificación, clínica, diagnóstico, y tratamiento de este tumor maligno de la confluencia biliar. En cuanto a su diagnóstico, se describen los signos directos e indirectos que deben tenerse en cuenta al realizar una ecografía, así como algunos aspectos del diagnóstico diferencial.