Advances in surgical treatment of hilar cholangiocarcinoma / 中华肝胆外科杂志

Hilar cholangiocarcinoma (HCCA) is the most common type of cholangiocarcinoma, with in sidious onset and a high degree of malignancy, and there are often lymph node metastasis and vascular invasion. R 0 resection by surgery is still the main treatment for cure. This article reviews the hot issues and their related advances in surgical treatment of hilar cholangiocarcinoma, including preoperative evaluation, surgical treatment and systematic treatment.

Research progress in the surgical treatment of hilar cholangiocarcinoma / 中华肝胆外科杂志

Hilar cholangiocarcinoma (HCCA) is a type of bile duct cancer with insidious onset and poor prognosis. Surgical resection is the only potentially curative treatment. The selection of surgical approaches and prognosis assessment depend on different classification systems and preoperative imaging results. This article reviews the progress in classification systems, imaging modalities, biliary drainage techniques, postoperative adjuvant therapy, future liver remnant insufficiency treatment, liver transplantation and resection extent for HCCA.

Patients with perihilar cholangiocarcinoma resection combined with portal vein resection and reconstruction / 中华肝胆外科杂志

Objective:To study the safety and efficacy of combining portal vein resection and reconstruction (PVR) with resection of perihilar cholangiocarcinoma (PHC).Methods:A total of 104 patients with PHC who underwent hepatectomies for either biliary resection alone or biliary resection combined with PVR from October 2006 to December 2019 at the Department of Hepatopancreatobiliary, Ningbo Medical Center of Lihuili Hospital entered into this study. There were 63 males and 41 females, with the age of (64.4±10.4) years. The control group consisted of 75 patients who underwent biliary resection alone, while the PVR group consisted 29 patients with biliary resection combined with PVR. The patient characteristics and the follow-up outcomes of the two groups were analyzed and compared. Survival analyses were performed using the Kaplan Meier method with the log-rank test.Results:Wedge resection of portal vein, side to side anastomosis in 2 cases, segmental resection and end to end anastomosis in 27 cases. The time taken for PVR and portal vein resection were (12.7±2.9)(range 8 to 18)min and (20.7±7.3)(range 8 to 38) mm, respectively. The estimated blood loss for the PVR group was significantly more than the control group [ M( Q1, Q3)] 800.0 (600.0, 1 500.0) ml vs. 600.0(500.0, 1 000.0) ml ( P<0.05). Based on postoperative pathological studies, the proportion of lymph node metastasis was significantly higher in the PVR group than the control group (58.6% vs. 32.0%, P<0.05). Clavien-Dindo grade Ⅲ and above complications were 30.7%(23/75) and 34.5%(10/29) in the control and PVR groups, respectively ( P>0.05). The re-operation and postoperative 90 days mortality rates were 9.3%(7/75) and 2.7%(2/75) in the control group, compared with 3.4%(1/29) and 0 in the PVR group, respectively (both P>0.05). The 1-, 3- and 5-year survival rates were 81.1%, 44.8% and 36.4% respectively for the control group and 78.1%, 35.9% and 31.4% for the PVR group (χ 2=0.33, P=0.570). Conclusion:When compared to biliary resection alone, biliary resection combined with PVR did not significantly increase postoperative complication and mortality rates, but with comparable long-term survival outcomes. Combined biliary resection with PVR was safe and improved the resection rate in selected patients with locally advanced PHC.

Whole disease cycle management of hilar cholangiocarcinoma / 中华肝胆外科杂志

Hilar cholangiocarcinoma is the most common malignant tumor of the biliary tract, the survival rate is poor due to the difficulty of early diagnosis and surgery. Progress have been made on hilar cholangiocarcinoma treatment during recent years, but it is still challenging to make a breakthrough. The whole disease cycle management is of great significance to improve the prognosis of patients with hilar cholangiocarcinoma. The whole disease cycle management refers to the whole-process scientific management including disease diagnosis, preoperative evaluation and preparation, surgical plan formulation and implementation, preoperative and postoperative adjuvant treatment and follow-up. This article summarized the domestic and foreign progress on the management of hilar cholangiocarcinoma in all stages of the whole disease cycle and shared the author's team's experience in the diagnosis and treatment of hilar cholangiocarcinoma.

Efficacy and safety of laparoscopic versus open radical resection for hilar cholangiocarcinoma: a meta-analysis / 中华肝胆外科杂志

Objective:This article aimed to systematically evaluate the safety, feasibility and short-term outcomes of laparoscopic versus open radical resection for hilar cholangiocarcinoma.Methods:Relevant medical literatures published before November 30, 2021 were searched using PubMed, EMbase, Cochrane Library, Web of Science, CNKI, Wanfang, VIP, CBM databases. Based on predetermined inclusion and exclusion criteria, articles were selected, and RevMan5.4 software was used to conduct the meta-analysis of this study.Results:Twelve comparative cohort articles were enrolled, which included 634 patients. There were 275 patients who underwent laparoscopic radical resection for hilar cholangiocarcinoma in the laparoscopic group, and 359 patients who underwent open radical resection for hilar cholangiocarcinoma in the open group. Meta-analysis showed that when compared with the open group, operation time of the laparoscopic group was significantly longer ( MD=58.36, 95% CI:13.54-103.18). However, intraoperative blood loss ( MD=-79.70, 95% CI:-90.01--69.39), hospital stay ( MD=-3.37, 95% CI:-4.29--2.45) and incision-related complications ( OR=0.36, 95% CI:0.16-0.80) were all significantly less (all P<0.05). The number of lymph node harvested, R 0 resection rates, postoperative bile leakage, intraperitoneal infection and effusion, total complication rates, and perioperative mortality and 1-year survival rates after operation between the two groups were not significantly different (all P>0.05). Conclusion:Laparoscopic radical resection for hilar cholangiocarcinoma was feasible and safe in selected patients carried out in an experienced hepatobiliary surgery center.

Short-term clinical outcomes of laparoscopic versus open radical resection for hilar cholangiocarcinoma / 中华肝胆外科杂志

Objective:To compare the short-term clinical outcomes of patients undergoing laparoscopic versus open radical resection of hilar cholangiocarcinoma.Methods:The clinical data of 91 patients who underwent radical resection for hilar cholangiocarcinoma at our hospital from January 2018 to June 2021 were analyzed retrospectively. There were 48 males and 43 females, with aged of (61.51±7.18) years old. The patients were divided into the laparotomy group ( n=59) and the laparoscopic group ( n=32) based on the operations they received. The general data, perioperative data and complications of the two groups were compared. Results:There was no perioperative death in the laparoscopic group, but one patient died of abdominal bleeding in the laparotomy group. All other patients recovered from postoperative complications with treatment. When compared with patients in the laparotomy group, the operation time [(381.28±102.37) vs. (296.81±84.74) min] and biliary intestinal anastomosis time [(17.81±2.81) vs. (15.19±2.27) min] were significantly longer in the laparoscopic group. However, the postoperative hospital stay [(12.34±3.46) vs. (15.10 ± 4.48) d], bed rest time [(3.38±0.66) vs. (5.24±0.88) d], analgesic time [(4.31±0.90) vs. (6.22±1.26) d] and postoperative time to first feeding [(3.91±0.89) vs. (5.32±0.86) d] were significantly lower ( P<0.05). There were no significant differences in amounts of intraoperative bleeding, numbers of lymph node harvested and incidences of postoperative complications between groups ( P>0.05). Conclusion:Under the premise of strictly indications, laparoscopic radical resection of hilar cholangiocarcinoma was safe and feasible, and had certain advantages in promoting the rapid recovery of patients.

Analysis of risk factors of postoperative biliary leakage in patients with perihilar cholangiocarcinoma / 中华肝胆外科杂志

Objective:To analyse the risk factors of biliary leakage after surgical resection in patients with perihilar cholangiocarcinoma (PHCC).Methods:The medical data on 179 patients who underwent surgical resection for PHCC at the Department of Hepatopancreatobiliary Surgery, Ningbo Medical Centre Lihuili Hospital, Ningbo University from April 2000 to April 2020 were collected, and 160 patients were finally enrolled into this study. There were 86 males and 74 females, aged (63.4±10.8) years. The 44 patients with class B biliary leakage and the 5 patients with class C biliary leakage were classified into the biliary leakage group, while the remaining 111 patients were classified into the control group. Risk factors of biliary leakage were analysed by univariate and multivariate logistic regression analyses.Results:Operation time ≥360 min, resection and reconstruction of hepatic hilar vessels on the preserved side of liver and number of bile duct openings of >3 in remnant liver were significantly higher in the biliary leakage than the control group (all P<0.05). Multivariate analysis showed that resection and reconstruction of hepatic hilar vessels on the preserved side ( OR=2.322, 95% CI: 1.078-5.002, P=0.028) and 3 or more bile duct openings in the remnant liver ( OR=2.656, 95% CI: 1.198-5.892, P=0.016) were significantly associated with biliary leakage. Conclusion:Resection and reconstruction of hepatic hilar vessels on the preserved side of liver and 3 or more bile duct openings in remnant liver were independent risk factors for biliary leakage after PHCC resection.

Application of 192Ir brachytherapy combined with external beam radiation and biliary stent in the treatment of unresectable hilar cholangiocarcinoma / 国际肿瘤学杂志

Objective:To evaluate the efficacy and safety of 192Ir brachytherapy combined with external beam radiation for the treatment of unresectable hilar cholangiocarcinoma. Methods:The clinical data of 26 patients with unresectable hilar cholangiocarcinoma admitted to Hebei Province Cangzhou Hospital of Integrated Traditional and Western Medicine from February 2016 to July 2018 were retrospectively analyzed. All patients received 192Ir brachytherapy combined with external beam radiation. First, percutaneous hepatobiliary stent implantation was given, followed by external beam radiotherapy (radiotherapy dose 45 Gy, 25 times), and then 192Ir brachytherapy (radiotherapy dose 20 Gy, 4 times), a total of 4 to 6 cycles of chemotherapy. The short-term and long-term efficacy were evaluated, and the Karnofsky performance status (KPS) score, CA19-9, total bilirubin (TBIL), direct bilirubin (DBIL), alanine aminotransferase (ALT), aspartate aminotransferase (AST) level changes and percutaneous transhepatic biliary drainage (PTCD) tube indwelling time were analyzed. Adverse reactions were recorded according to the Radiation Therapy Oncology Group (RTOG) injury classification standard. Results:Three months after the end of treatment, 4 cases (15.4%) were completely remitted, 20 cases (76.9%) were partially remitted, 2 cases (7.7%) were stable, and there was no disease progression. The objective remission rate was 92.3% (24/26). The 1-year and 2-year local control rates were 75.0% and 62.5% respectively, and the 1-year and 2-year overall survival rates were 57.7% and 26.9% respectively. The KPS score was 70.39±10.76 one month after the treatment, which was significantly higher than the 60.00±10.58 before treatment ( t=-27.00, P<0.001). The levels of CA19-9 before treatment and 1 month, 2 months and 3 months after treatment were (390.88±202.62) U/ml, (322.45±204.06) U/ml, (254.00±160.49) U/ml, (182.85±124.05) U/ml, which showed a gradual decrease trend, and there was a statistically significant difference ( F=126.94, P<0.001). TBIL [(250.88±80.83) μmol/L, (153.98±61.74) μmol/L, (93.45±38.12) μmol/L, (53.82±26.75) μmol/L], DBIL [(205.82±66.68) μmol/L, (133.23±58.53) μmol/L, (64.31±36.25) μmol/L, (40.55±26.16) μmol/L], ALT [(163.92±54.12) U/L, (68.23±28.86) U/L, (45.73±21.94) U/L, (32.66±12.88) U/L], AST [(177.69±58.68) U/L, (79.23±32.87) U/L, (49.77±25.45) U/L, (35.54±16.10) U/L] showed progressive decline, with statistically significant differences ( F=315.60, P<0.001; F=385.30, P<0.001; F=284.24, P<0.001; F=311.80, P<0.001), and liver function was improved. The PTCD tube was removed after treatment in all patients, with a median time of 54 days (49-96 days). During the treatment, bone marrow suppression, nausea and vomiting, abdominal pain and biliary tract infection occurred. All of them improved after symptomatic treatment. No serious complications such as bile leakage and biliary hemorrhage occurred. Conclusion:192Ir brachytherapy combined with external beam radiation has a reliable curative effect in the treatment of unresectable hilar cholangiocarcinoma. It can improve the quality of life of patients, and the adverse reactions can be tolerated. It provides a feasible and safe treatment method for the clinic.

Application of ultra-fine metal stents to malignant hilar biliary strictures / 中华消化内镜杂志

Objective:To evaluate the feasibility, efficacy, and safety of ultra-fine metal stents for malignant hilar biliary strictures (MHBS).Methods:Ultra-fine metal stents were simultaneously placed into the intrahepatic duct of 30 patients with unresectable malignant hilar biliary strictures of Bismuth type Ⅱ or higher (based on imaging or histological and/or cytological findings) by using the stent-by-stent mode from January 2014 to June 2021 at the Department of Gastroenterology, Eastern Hepatobiliary Surgical Hospital. The technical success rate, clinical efficacy and incidence of complications were observed.Results:The technical success rate was 100.0% (30/30), and the clinical success was achieved in 28 patients (93.3%) . The mean procedure time was 55.7±20.7 min and the placement time was 28.3±18.2 min. Early adverse events included mild acute pancreatitis ( n=2) and cholangitis ( n=5). The median stent patency and the median overall survival were 243 days (95% CI: 186.6-299.4 days) and 237 days (95% CI: 149.0- 325.0 days), respectively. The incidence of late cholangitis was 36.7% (11/30). Stent malfunction occurred in 14 of the 30 patients, and 5 of them received endoscopic reintervention. The technical success rate for the reintervention was 4/5 with the mean procedure time of 49.8±6.9 min. Conclusion:Malignant hilar biliary obstruction endoscopic palliation with ultra-fine metal stents using the stent-by-stent mode is safe, feasible and effective.

Application of a new plastic biliary stent modified based on pigtail nasal bile drainage tube to hilar biliary cholangiocarcinoma / 中华消化内镜杂志

Objective:To compare the efficacy and safety of a new type of plastic biliary stent modified based on the pigtail nasobiliary duct and the common plastic biliary stent for hilar cholangiocarcinoma.Methods:Data of a total of 38 patients with obstructive jaundice caused by hilar cholangiocarcinoma who received endoscopic retrograde cholangiopancreatography (ERCP) palliative treatment at the Endoscopy Center, General Hospital of Northern Theater Command from June 2018 to December 2020 were collected, including 20 cases using the new type of plastic biliary stent (the new stent group), and 18 cases using the common plastic stent (the common stent group). Patients were followed up to May 30, 2021. The procedure time, hospital stay, postoperative biliary infection incidence, the bilirubin decrease, and the patency time of the stents in the two groups were compared.Results:The procedure time was 19.85±1.07 minutes in the new stent group and 22.00±3.38 minutes in the common stent group, respectively, showing no significant difference between them ( t=1.26, P=0.607). The lengths of hospital stay of the two groups were 11.45±2.39 days and 11.33±3.51 days, respectively, showing no significant difference between them ( t=-0.52, P=0.938). The median margins of total bilirubin reduction in the two groups were 122.85 μmol/L and 96.25 μmol/L, respectively, with significant difference ( Z=-2.03, P=0.042). The incidence of long-term cholangitis of the new stent group was significantly lower than that of the common stent group [10.0% (2/20) VS 44.4% (8/18), P=0.027]. The patency time of the new stent group was significantly longer than that of the common stent group (109.45±32.67 days VS 82.11±20.95 days) with significant difference ( t=2.23, P=0.032). Conclusion:In the palliative treatment of hilar bile duct obstruction, the new plastic bile duct stent modified based on pigtail type can reduce the incidence of long-term cholangitis and prolong the patency of bile duct stent compared with the common stent group.

Technical points of combined vascular rsection and reconstruction in radical hilar cholangiocarcinoma / 中华肝胆外科杂志

Hilar cholangiocarcinoma (HCCA) is a highly malignant tumor adjacent to liver tissue and the anatomical site of the hepatic portal, which easily invades the hepatic arteries and portal veins. Consequently, vascular resection and reconstruction are significant for the surgical treatment of HCCA, which are the key to improving the R 0 resection rate and long-term efficacy. The resection and reconstruction of hepatic hilar vessels, especially the hepatic arteries, have always been a challenge for surgeons. The purpose of this article is to discuss the surgical points of HCCA radical treatment combined with resection and reconstruction of portal vein and hepatic artery to help clinicians improve the R 0 resection rate of HCCA, thus improving the outcomes and prognosis of patients.

Open surgery in the treatment of patients with hilar cholangiocarcinoma: a study on curative efficacy and prognostic factors / 中华肝胆外科杂志

Objective:To study the efficacy of different surgical methods in treatment of hilar cholangiocarcinoma (HCCA), and to analyze the factors affecting long-term prognosis of HCCA patients after surgical treatment.Methods:The clinical data of 105 patients who underwent surgical treatment for HCCA at the First Affiliated Hospital of Soochow University from April 2010 to October 2021 were retrospectively analysed. There were 58 males and 47 females, with age (64.2±10.6) years old. Data analysed included surgical treatments, postoperative pathological data, perioperative complications and survival on follow-up. The Kaplan Meier survival curve was plotted, and the log-rank test and Cox proportional hazard model were performed to analyze the key factors affecting long-term prognosis.Results:Of 105 patients, 4 (3.8%) patients died during the perioperative period, and 58 patients (55.2%) developed complications with included 32 (30.5%) patients with pleural effusion and 12 (11.4%) patients with biliary leakage. The follow-up data was available in 85 patients with the overall median survival time of 19 months, and the 1-, 3-, 5-year cumulative survival rates of 58.1%, 27.0% and 24.8% respectively. The 1-, 3-, and 5-year cumulative survival rates for the R 0 resection patients ( n=59) were 69.4%, 36.2%, 27.4%, respectively, which were significantly better than 49.4%, 12.3%, 0% for the R 1/2 resection patients ( n=20), and 0% for the palliative drainage patients ( n=6) (all P<0.05). Univariate analysis and Cox multivariate analysis showed that age ≥70 years ( HR=2.158, 95% CI: 1.175-3.965), preoperative CA19-9 level ≥1 015 U/ml ( HR=1.981, 95% CI: 1.009-3.894), resection margin ( HR=2.587, 95% CI: 1.371-4.881), and lymph node metastasis ( HR=2.308, 95% CI: 1.167-4.567) were independent risk factors for long-term prognosis of HCCA patients (all P<0.05). Conclusions:R 0 resection was an effective way to prolong survival of patients with HCCA. Age, preoperative CA19-9 level, resection margin and lymph node metastasis were related to long-term survival of HCCA patients.

A clinical study using a new approach for laparoscopic radical resection of Bismuth-Corlette Ⅲa hilar cholangiocarcinoma / 中华肝胆外科杂志

Objective:To study the safety and feasibility of laparosocpic left-liver-first anterior radical modular orthotopic right hemihepatectomy (Lap-Larmorh) in treatment of Bismuth-Corlette type Ⅲa hilar cholangiocarcinoma.Methods:From June 2020 to April 2022, 13 patients with Bismuth-Corlette type Ⅲa hilar cholangiocarcinoma underwent Lap-Larmorh at the Department of Hepatobiliary Surgery of the Second Hospital of Hebei Medical University. There were 8 males and 5 females, aged (60.9±8.4) years. The operation time, intraoperative blood loss, complications and postoperative survival were analyzed.Results:Five patients were treated with percutaneous transhepatic cholangial drainage to reduce jaundice before surgery. All 13 patients successfully underwent the Lap-Larmorh and there was no conversion to laparotomy. The operative time was [ M( Q1, Q3)] 390.0 (355.0, 435.0) minutes, and the intraoperative blood loss was [ M( Q1, Q3)] 800.0 (300.0, 1 100.0) ml. Postoperative complications occurred in 4 patients with pleural effusion, and 1 patient had portal vein thrombosis associated with pleural effusion, who was managed successfully using low molecular weight heparin sodium anticoagulation therapy and pleural effusion tapping. The postoperative hospital stay was (12.5±5.5) days, and there was no perioperative death. Intraoperative frozen pathology showed 12 patients with left hepatic duct with negative margins, and 1 patient with moderate dysplasia. Postoperative paraffin pathology showed 12 patients with bile duct adenocarcinoma, 1 patient with bile duct mucinous adenocarcinoma, 2 patients with tumors of high differentiation, 10 patients with moderate differentiation, and 1 patient with poor differentiative. The R 0 resection rate was 100.0% (13/13) and the tumor diameter was (2.9±0.9) cm. Follow-up by telephone or outpatient clinics after operation showed 1 patients to be lost to follow-up. The 1-year survival rate after operation was 66.7% (8/12). Conclusion:For Bismuth-Corlette type Ⅲa hilar cholangiocarcinoma, the new laparoscopic approach of Lap-Larmorh was safe and feasible.

Selection of surgical treatment for Bismuth-Corlette type III and IV hilar cholangiocarcinoma / 中华肝胆外科杂志

Objective:To analyze the efficacy and prognosis of different surgical treatments for Bismuth-Corlette type III and IV hilar cholangiocarcinoma (HCCA).Methods:The clinical data of 86 Bismuth-Corlette type III and IV HCCA patients treated at the First Affiliated Hospital of Anhui Medical University from January 2010 to December 2016 were retrospectively analyzed. There were 45 males and 41 females with age of (59.5±10.5) years old. According to the operative method, 57 patients were included into the extended hepatectomy group, and 29 patients into the perihilar hepatectomy group. The perioperative clinical data and survival rates were compared between the two groups. Through inpatient interviews, regular outpatient or telephone follow-up, factors affecting prognosis were analyzed by univariate and multifactorial Cox regression.Results:The operative time and intraoperative blood loss in the extended hepatectomy group were significantly higher than those in the perihilar hepatectomy group, [320(270, 380) min vs. 270(210, 300) min, P<0.001; 300(200, 400) ml vs. 100(100, 150) ml, respectively P<0.001]. The incidences of ≥ Clavien-Dindo grade III complications and ISGLS grade C liver failure in the extended hepatectomy group were significantly higher than those in the perihilar hepatectomy group [36.4%(20/57) vs. 13.8% (4/29), P=0.037; 13.8% (7/57) vs. 0(0/29), respectively P=0.047]. The cumulative 1-, 3- and 5-year survival rates of the extended hepatectomy group were 89.5%, 38.6% and 19.3%, respectively. The cumulative 1-, 3- and 5-year survival rates of perihilar hepatectomy group were 86.2%, 20.7% and 10.3%, respectively. The difference between the two groups was statistically significant ( P=0.048). Multivariate analysis showed that perihilar hepatectomy ( HR=1.958, 95% CI: 1.174-3.268, P=0.010), non-R 0 resection ( HR=6.040, 95% CI: 2.915-12.513, P<0.001) and TNM stage III/IV( HR=2.144, 95% CI: 1.257-3.654, P=0.005) were independent risk factors for overall survival after surgery for HCCA patients ( P<0.01). Conclusions:Patients with Bismuth-Corlette type III and IV HCCA who received extended hepatectomy had significantly better overall survival than those patients who underwent perihilar hepatectomy. However, the incidences of surgical complications and liver failure in the extended hepatectomy group were also significantly higher.

Tumor de Klatskin en una paciente con diagnóstico de síndrome de Sjögren / Klatskin tumor in a patient diagnosed with Sjögren's syndrome

El proceso inflamatorio crónico de las enfermedades reumáticas constituye un factor que causa afectación en otros órganos y sistemas de órganos. La afectación puede llegar a producir metaplasia de la mucosa de distintos tejidos y condicionar la aparición de enfermedades neoplásicas. Los pacientes con síndrome de Sjögren tienen un 40 por ciento de aumento de riesgo de padecer una enfermedad tumoral con respecto a los pacientes sanos o con otras enfermedades crónicas no inflamatorias. El objetivo del presente estudio es dar a conocer las características clínico-imagenológicas que posibilitaron el diagnóstico de un tumor de Klatskin en una paciente femenina de 57 años de edad con antecedentes de 5 años de evolución del síndrome de Sjögren. Se realizó una colangiopancreatografía retrógrada endoscópica para eliminar el tumor. A los tres días de la intervención quirúrgicamente la paciente muestra una evolución favorable por lo que se decide dar alta hospitalaria con seguimiento por consulta externa de especialidades de cirugía general y reumatología indistintamente. Durante todo el procedimiento se mantuvo el tratamiento para la enfermedad de base de la paciente. Actualmente la paciente se encuentra incorporada a sus actividades cotidianas y con una evolución favorable de su estado de salud(AU)

The chronic inflammatory process of rheumatic diseases constitutes a factor that causes affectation in other organs and organ systems. The affectation can produce metaplasia of the mucosa of different tissues and condition the appearance of neoplastic diseases. Patients with Sjögren's syndrome have a 40 percent increased risk of developing a tumor disease compared to healthy patients or other non-inflammatory chronic diseases. The objective of this study is to present the clinical-imaging characteristics that made the diagnosis of a Klatskin tumor possible in a 57-year-old female patient with a 5-year history of Sjögren's syndrome. Endoscopic retrograde cholangiopancreatography was performed to remove the tumor. Three days after the surgical intervention, the patient showed a favorable evolution, so it was decided to discharge from the hospital with follow-up by outpatient consultation of specialties of general surgery and rheumatology indistinctly. Treatment for the patient's underlying disease was maintained throughout the procedure. Currently the patient is incorporated into her daily activities and with a favorable evolution of her state of health(AU)

Neurofibroma de la vía biliar: una causa rara de ictericia colestásica que imita el tumor de Klatskin

El neurofibroma de la vía biliar es una enfermedad extremadamente rara y generalmente asintomática, sin embargo, puede ocasionar ictericia obstructiva y simular un tumor de Klatskin conduciendo a un tratamiento quirúrgico radical y mayor morbilidad del paciente. Presentamos el caso de una mujer de 62 años colecistectomizada hace 10 años, con ictericia y dolor en hipocondrio derecho, así como exámenes auxiliares compatibles con colestasis. Se observó dilatación de la vía biliar y presencia de un tumor en el conducto hepático izquierdo mediante colangioresonancia magnética. El diagnóstico clínico preoperatorio fue Colangiocarcinoma hiliar, pero el estudio histopatológico reveló una neoplasia compuesta por células fusocelulares sin atipia ni actividad mitótica, cuya estirpe neurogénica se sustentó por su positividad a proteína S100 en inmunohistoquímica. Reportamos el caso dada su poca frecuencia en la literatura y su relevancia, al ser una entidad benigna, como diagnóstico diferencial de cáncer.

The neurofibroma of the bile duct is an extremely rare and generally asymptomatic disease, however, it maybe cause obstructive jaundice and mimic a Klatskin tumor, leading to radical surgical treatment and increased patient morbidity. We present the case of a 62-year-old woman who underwent cholecystectomy 10 years ago, with jaundice and pain in the right upper quadrant, as well as auxiliary tests compatible with cholestasis. Dilation of the bile duct and the presence of a tumor in the left hepatic duct were observed by magnetic cholangioresonance. The preoperative clinical diagnosis was hilar cholangiocarcinoma, but the histopathological study revealed a neoplasm composed of spindle cells without atypia or mitotic activity, whose neurogenic lineage supported by its positivity to protein S100 in immunohistochemistry. We report the case given its infrequency in the literature and its relevance, as it is a benign entity, as a differential diagnosis of cancer.

Protocol for liver transplantation in hilar cholangiocarcinoma / Protocolo de transplante hepático para colangiocarcinoma hilar

ABSTRACT Background: Hilar cholangiocarcinoma represents more than half of all cholangiocarcinoma cases, having poor prognosis and presenting a median overall survival after diagnosis of 12-24 months. In patients who have unresectable tumors with a better prognosis, the proposal to perform liver transplantation emerged for expanding the possibility of free margins by performing total hepatectomy. Aim: To provide a Brazilian protocol for liver transplantation in patients with hilar cholangiocarcinoma. Method: The protocol was carried out by two Brazilian institutions which perform a large volume of resections and liver transplantations, based on the study carried out at the Mayo Clinic. The elaboration of the protocol was conducted in four stages. Result: A protocol proposal for this disease is presented, which needs to be validated for clinical use. Conclusion: The development of a liver transplantation protocol for cholangiocarcinoma aims not only to standardize the treatment, but also enable a better assessment of the surgical results in the future.

RESUMO Racional: O colangiocarcinoma hilar representa mais da metade de todos os casos de colangiocarcinoma; tem prognóstico reservado e sobrevida global mediana de 12- 24 meses após o diagnóstico. A proposta de realizar transplante hepático surgiu para ampliar a possibilidade de margens livres através de hepatectomia total nos portadores de tumoresirressecáveis com melhor prognóstico. Objetivo: Apresentar protocolo brasileiro para realização de transplante hepático em pacientes com colangiocarcinoma hilar. Método: O protocolo foi realizado por duas instituições com grande volume de ressecções e transplantes hepáticos no Brasil, baseado no trabalho realizado pela MayoClinic. A elaboração foi dividida em quatro etapas. Resultado: É apresentada proposta de protocolo para esta doença a ser validada na aplicação clínica. Conclusão: Foi possível elaborar protocolo de transplante hepático para colangiocarcinoma a fim de uniformizar o tratamento e melhor avaliar os resultados cirúrgicos.

Research advances in surgical margin of hilar cholangiocarcinoma / 临床肝胆病杂志

Hilar cholangiocarcinoma (HCCA) is the most common biliary malignancy, and surgical operation is the only possible treatment method at present. The nature of surgical margin is an important influencing factor for the long-term survival of patients, and radical resection can bring great survival benefits to patients. The determination of radial margin for HCCA can help to evaluate the nature of surgical margin and predict the prognosis of patients more accurately. This article reviews the latest research advances in the surgical margin of HCCA.

Application of accurate endoscopic retrograde cholangiopancreatography drainage to preoperative jaundice reduction for hilar cholangiocarcinoma / 中华消化内镜杂志

Objective:To investigate the application of accurate endoscopic retrograde cholangiopancreatography (ERCP)drainage guided by IQQA ?-Liver CT(an image interpretation and analysis system)to preoperative jaundice reduction for hilar cholangiocarcinoma. Methods:Data of 12 patients with hilar cholangiocarcinoma who planned to receive surgical treatment in the hepatobiliary surgery department of Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School from May 2017 to December 2018 were reviewed. All patients were treated with ERCP drainage before surgery. The procedure success rate, postoperative drainage volume and postoperative complications of the patients were analyzed.Results:ERCP was successful in all patients, and the median daily volume of endoscopic nasobiliary drainage was 675 mL(400-1 500 mL). Only 1 patient developed postoperative cholangitis, and another patient developed postoperative mild pancreatitis. The mean serum total bilirubin was 173.3±62.8 μmol/L in the patients before ERCP, which decreased to 35.6±13.9 μmol/L before surgery.Conclusion:Accurate ERCP drainage can effectively reduce the serum bilirubin level in patients with hilar cholangiocarcinoma and reduce the incidence of cholangitis after the procedure, creating favorable conditions for subsequent surgical operations.

Risk factors of pathological positive resection margins in patients undergoing curative-intent resection for advanced hilar cholangiocarcinoma / 中华肝胆外科杂志

Objective:To study the risk factors of positive surgical margins in patients with advanced hilar cholangiocarcinoma (HCCA) undergoing curative-intent resection with the aim to provide references for clinical decision-making.Methods:The clinical pathological data of 126 patients with advanced HCCA who underwent curative-intent resection at the Chinese PLA General Hospital from January 2011 to December 2014 was retrospective analyzed. There were 78 males and 48 females, with an average age of 55 years. The patients were divided into two groups: the resection margin positive group (positive for residual tumor at any surgical margin, n=29) and the negative resection margin group ( n=97). Multivariate logistic regression analysis, in reference to the results of univariate analysis, was applied to the relevant variables to study independent risk factors of positive resection margin. Receiver operating characteristic (ROC) curves were drawn to evaluate the independent and joint predictive values of the relevant indicators. Results:Univariate analysis showed vascular involvement, tumor diameter volume ≥20.94 cm 3, plasma fibrinogen ≥3.36 g/L, and DRR≤0.61 were associated with positive resection margins (all P<0.05). A history of cholecystectomy was potentially associated with positive resection margins ( P<0.1). These variables were included in the multivariate logistic regression analysis which showed vascular involvement ( OR=4.134, 95% CI: 1.545-11.060, P=0.005), tumor size ≥ 20.94 cm 3 ( OR=2.926, 95% CI: 1.107-7.733, P=0.030) and DRR≤0.61 ( OR=3.170, 95% CI: 1.126-8.928, P=0.029) were independent risk factors of positive margins after curative resection in patients with advanced HCCA. ROC curve analysis was used to evaluate the impact of the combination of the above variables in predicting positive surgical margins. Sensitivity and specificity were calculated to be 86.2% and 65.7% respectively, and the area under the curve was 0.771. Conclusion:Vascular involvement, tumor size ≥20.94 cm 3 and DRR≤0.61 were independent risk factors of positive surfical margins in patients with advanced HCCA undergoing curative-intent resection. The combination of the above predictive indicators provided some references for treatment decisions.