The effect of prealbumin on the long-term prognosis of hilar cholangiocarcinoma following radical surgery / 中华外科杂志

Objective: To investigate the association between prealbumin and the long-term prognosis of patients with hilar cholangiocarcinoma(HCCA) following radical surgery. Methods: The clinical data of 262 HCCA patients who underwent radical surgery admitted from January 2010 to January 2017 at the First Affiliated Hospital of Army Medical University were collected,retrospectively. There were 158 males and 104 females; aged (57.6±9.9)years old(range:32 to 78 years). According to the preoperative serum prealbumin level(170 mg/L),the patients were divided into low prealbumin group(n=143) and normal prealbumin group(n=119). Follow-up until September 2020,the main research indicator was overall survival(OS), and the secondary research indicator was recurrence-free survival(RFS). The measurement data conforming to the normal distribution adopted the t test,the measurement data not conforming to the normal distribution adopted the Mann-Whitney U test,and the count data adopted the χ2 test. The Kaplan-Meier method was used to calculate the cumulative survival rate. The Log-rank test was used for univariate analysis of the cumulative survival rate. Variables with P<0.10 in univariate analysis were included in the Cox proportional hazards model for multivariate analysis. Results: The 1-, 3-, and 5-year OS rate of the 262 patients was 73.4%, 32.1%, and 24.0%, respectively, and the 1-, 3-, and 5-year RFS rate was 54.6%, 25.2%, and 16.2%, respectively. Median OS and RFS were 21 months and 12 months for patients with low prealbumin and 25 months and 19 months for patients with normal prealbumin. The OS rate and RFS rate of patients in the low prealbumin group were lower than those in the normal prealbumin group, and the difference was statistically significant (both P<0.05). The results of univariate analysis indicated that low prealbumin, CA19-9>150 U/L, tumor infiltration length>3 cm, preoperative jaundice, macrovascular invasion, microvascular invasion, lymph node metastasis, and poor differentiation maybe the risk factors of OS,and low prealbumin,tumor invasion length>3 cm,macrovascular invasion, microvascular invasion,lymph node metastasis,and poor differentiation maybe the risk factors of RFS for postoperative for radical resection in patients with HCCA (all P<0.10). Multivariate results suggested that low prealbumin,tumor invasion length>3 cm,microvascular invasion,lymph node metastasis,and poor differentiation were independent risk factors affecting OS and RFS in patients with HCCA after radical operation (all P<0.05). Conclusion: Preoperative prealbumin level can predict the long-term prognosis of patients with hilar cholangiocarcinoma following radical surgery.

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)

Colangiocarcinoma, diagnóstico, tratamiento y evolución clínica. Presentación de un caso con sobrevida 5 años / Cholangiocarcinoma: diagnosis, treatment and clinical evolution. Presentation of a case with five-year survival

RESUMEN El colangiocarcinoma es un tumor maligno originado en el epitelio de los conductos biliares intra o extrahepáticos. En el cuadro clínico destacan el dolor en hipocondrio derecho, ictericia y baja de peso. Actualmente, el diagnóstico se ha facilitado por la disponibilidad de variados procedimientos imagenológicos y endoscópicos. Se presentó un caso al que se le realizó el diagnóstico de este tipo de tumor. Se sometió a tratamiento endoscópico, quirúrgico y oncológico con Gemcitabina, Cisplatino y Oxaliplatino. Fue seguido por equipo multidisciplinario y evolucionó con sobrevida de 5 años (AU).

ABSTRACT Cholangiocarcinoma is a malignant tumor originated in the epithelium of the intra or extra hepatic biliary ducts. Pain in the right hypochondrium, jaundice and low weight are the main clinical features. Currently, the diagnosis has been facilitated by the availability of different imaging and endoscopic procedures. The authors presented a case diagnosed with this kind of tumor. The patient underwent surgical, endoscopic and oncologic treatment with gemcitabine, cisplatine and oxaliplatine. He was followed up by a multidisciplinary team and evolved with five-year survival (AU).

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.

Elementos etiopatogénicos y diagnósticos del tumor de Klatskin o colangiocarcinoma hiliar / Etiopathogenetic and Diagnostic Elements of Klatskin's Tumor or Hilar Cholangiocarcinoma

RESUMEN Introducción: El colangiocarcinoma hiliar es un tumor poco frecuente, de mal pronóstico y elevada mortalidad; con un curso silente hasta la fase avanzada de la enfermedad. Objetivo: Describir la etiopatogenia y el diagnóstico por imágenes del colangiocarcinoma hiliar. Métodos: De las bases datos PubMed, SciELO y Latindex, se seleccionaron artículos publicados desde 2005 hasta mayo de 2020, relacionados con el colangiocarcinoma hiliar/ perihiliar: etiopatogenia, diagnóstico clínico, estudios de laboratorio y estudios imaginológicos. Desarrollo: Ictericia obstructiva (90 por ciento), pérdida de peso (60 - 75 por ciento) y dolor abdominal (40 por ciento) constituyen los síntomas de presentación más frecuentes. Los factores predisponentes, genéticos y ambientales, desencadenan respuesta inflamatoria crónica que lesionan el DNA de las células ductales provocando diferenciación celular anómala con el desarrollo de colangiocarcinoma. Las infecciones parasitarias y enfermedad litiásica de las vías biliares, en países orientales y la colangitis esclerosante primaria, en occidente, constituyen los principales factores predisponentes. El trípode para el diagnóstico lo conforman: la ecografía, tomografía axial computarizada y la resonancia magnética nuclear. Conclusiones: El colangiocarcinoma hiliar es una causa de colestasis poco frecuente en la población general, con un predominio sexta década de la vida y en el sexo masculino, siendo la ictericia el motivo de consulta de estos pacientes, donde la ecografía y la tomografía axial computarizada juegan el papel más importante en su diagnóstico y donde la clasificación de Bismuth-Corlette y TNM son esenciales para la correcta planificación del tratamiento(AU)

ABSTRACT Introduction: Hilar cholangiocarcinoma is a rare tumor, with poor prognosis and high mortality, with a silent course until the advanced stage of the disease. Objective: To describe the etiopathogenesis and imaging diagnosis of hilar cholangiocarcinoma. Methods: From the PubMed, SciELO and Latindex databases, articles published from 2005 to May 2020 were selected, insofar they were related to hilar/perihilar cholangiocarcinoma: etiopathogenesis, clinical diagnosis, laboratory studies and imaging studies. Development: Obstructive jaundice (90 percent), weight loss (60-75 percent) and abdominal pain (40 percent) are the most frequent presenting symptoms. Predisposing factors, either genetic and environmental, trigger chronic inflammatory responses that damage the DNA of ductal cells, causing abnormal cell differentiation with the development of cholangiocarcinoma. Parasitic infections and bile duct stone disease in Eastern countries and primary sclerosing cholangitis in the West are the main predisposing factors. The tripod for diagnosis is made up of ultrasound, computerized axial tomography and nuclear magnetic resonance. Conclusions: Hilar cholangiocarcinoma is a rare cause of cholestasis among the general population, with a predominance during the sixth decade of life and among males, jaundice being the reason for consultation of these patients, in which ultrasound and computerized axial tomography play the most important elements for its diagnosis, while the TNM and Bismuth-Corlette classification are essential for the correct planning of treatment(AU)

Tumor de Klatskin (colangiocarcinoma hiliar) su morbimortalidad en un servicio de cirugía / Klatskin's tumor (hilar cholangiocarcinoma) his morbi-mortality in a surgery service

RESUMEN Introducción: El tumor de Klatskin es el colangiocarcinoma, más frecuente de la vía biliar siendo responsable de una alta morbimortalidad en los servicios de cirugía. Objetivo: Determinar la morbilidad y la mortalidad por tumor de Klatskin en el Servicio de Cirugía del Hospital Universitario "Manuel Ascunce Domenech". Métodos: Se realizó un estudio descriptivo, prospectivo y observacional de pacientes que ingresaron en el Servicio de Cirugía General con diagnóstico de tumor de Klatskin, entre septiembre de 2018 y enero del 2020. El universo estuvo conformado por 7 pacientes que cumplieron con los criterios de inclusión. Se utilizaron métodos estadísticos descriptivos y cálculos con valores porcentuales. Resultados: La mayor incidencia de los pacientes fue de sexo masculino y de raza blanca, con un 71,4 por ciento y 85,7 por ciento respectivamente. Predominó adenocarcinoma como variedad histológica con un 85,7 por ciento. Tipo II de la clasificación topográfica, el procedimiento de Hess como operación realizada y la bilirragia como complicación prevaleció con un 42,8 por ciento respectivamente. El 85,7 por ciento de los pacientes egresaron vivos y con una cirugía con finalidad curativa. Conclusiones: La mayoría de los pacientes eran masculinos y de color blanco. Más de la mitad de los pacientes fueron clasificados como tipo I y II según clasificación de Bismuth-Corlette. El proceder de Hess, el adenocarcinoma como forma histológica y el estado del egreso vivo predominó en el total de pacientes(AU)

ABSTRACT Introduction: Klatskin's tumor is cholangiocarcinoma, most frequent to occur in the bile duct, being responsible for high morbidity and mortality in surgery departments. Objective: To determine the morbidity and mortality of Klatskin's tumor at the surgery service of Manuel Ascunce Domenech University Hospital. Methods: We carried out a descriptive, prospective and observational study of patients admitted to the general surgery service with a diagnosis of Klatskin's tumor, between September 2018 and January 2020. The study population consisted of seven patients who met the inclusion criteria. Descriptive statistical methods and calculations with percentage values were used. Results: The highest incidence was represented male and white patients, accounting for 71.4 percent and 85.7 percent, respectively. Adenocarcinoma predominated as a histological variety, accounting for 85.7 percent. There was prevalence of type II of topographic classification, the Hess procedure as the performed operation, and bilirrhagia as a complication, accounting for 42.8 percent, respectively. 85.7 percent of the patients were discharged and received surgery for curative purposes. Conclusions: Most of the patients were male and white. More than half of the patients were classified as types I and II, according to the Bismuth-Corlette classification. The Hess procedure, adenocarcinoma as a histological form, and discharge predominated in all patients(AU)

Tumor de Klatskin y metástasis cerebral como presentación inusual en cáncer de mama / Klatskin tumor and brain metastasis in a breast cancer patient: Case report

Introducción: El cáncer de mama es la principal causa de muerte en mujeres a nivel mundial, se los clasifica en base a la expresión del receptor de estrógenos, de progesterona y de ERBB2. El tumor de Klatskin es infrecuente y se origina en la bifurcación del conducto biliar extrahepático. Presentación del caso: Paciente femenina de 60 años con diagnóstico de cáncer de mama estadío IIIB, que presenta hemiplejía derecha secundaria a lesión cerebral metastásica, inicialmente catalogada como neurocisticercosis. Después de 1 año 7 meses desarrolla ictericia colestásica cuya evaluación clínica permite evidenciar tumor de Klatskin mediante colangioresonancia. Discusión: El presente caso muestra una paciente con múltiples metástasis secundarias a cáncer de mama. Incluyendo, de igual manera, un tumor de Klatskin, el cual fue descubierto posterior a un síndrome colestásico. Son pocos los casos de la literatura que registran procesos metastásicos hacia conductos biliares con origen en un carcinoma ductal infiltrante. Conclusiones: Pocos casos en la literatura registran procesos metastásicos hacia las vías biliares con origen en infiltración ductal carcinoma; sin embargo, es fundamental informar para la evidencia clínica y el estudio de la literatura.

Introduction: Breast cancer is the principal cause of death in women worldwide, classi ed based on the expression of estrogen receptor, progesterone, and ERBB2. Klatskin tumor is rare and originates in the bifurcation of the extrahepatic bile duct. Case presentation: A 60 years old female patient diagnosed with stage IIIB breast cancer presents right hemiplegia secondary to metastatic brain injury, initially classi ed as neurocysticercosis. After one year and seven months, she developed cholestatic jaundice, whose clinical evaluation allows Klatskin tumor evidence through cholangioresonance. Discussion: The present case is about a patient with multiple metastases secondary to breast cancer. Including, in the same way, a Klatskin tumor, which was discovered after a cholestatic syndrome. Conclusions: Few cases in the literature register metastatic processes towards bile ducts originating from in ltrating ductal carcinoma; however, it is essential to report for clinical evidence and study of the literature.

Application of 3D visualization and 3D printing in individualized precision surgery for Bismuth-Corlette type Ⅲ and Ⅳ hilar cholangiocarcinoma / 南方医科大学学报

OBJECTIVE@#To explore the application of 3D visualization and 3D printing in individualized precision surgical treatment of Bismuth-Corlette type Ⅲ and Ⅳ hilar cholangiocarcinoma.@*METHODS@#We retrospectively analyzed the data of 10 patients with hilar cholangiocarcinoma undergoing surgeries under the guidance of 3D visualization and 3D printing in the Department of Hepatobiliary Surgery, Zhujiang Hospital from May 2016 to March 2019. Thin-section CT data of the patients were collected for 3D reconstruction and 3D printing, and the 3D printed models were used for observing the 3D relationship of tumor with the intrahepatic bile duct, hepatic artery, portal vein and hepatic vein system and for performing preoperative simulated surgery and surgical planning. The 3D printed models were subsequently used for real-time intraoperative navigation to guide surgeries in the operating room.@*RESULTS@#3D visualization models were successfully reconstructed for all the 10 patients and printed into 3D models. The 3D visualization types in Bismuth-Corlette classification included type Ⅲa (4 cases), type Ⅲb (4 cases), and type Ⅳ (2 cases); 4 patients showed portal vein variation, 3 had hepatic artery variation, and 2 had both portal vein and hepatic artery variations. Two patients were found to have trifurcation type of portal vein variation, one had "I-shaped" variation, and one showed the absence of the right anterior branch of the portal vein; 3 patients had hepatic artery variations with the left hepatic artery originating from the left gastric artery (1 case) and the right hepatic artery originating from the superior mesenteric artery (2 cases). Four patients with type Ⅲb underwent left hepatectomy; 4 with type Ⅲa received right hepatectomy; 1 patient with of type Ⅳ received peripheral hepatic resection and another underwent left hepatectomy. The results of preoperative 3D reconstruction, 3D printed model and preoperative planning were consistent with the intraoperative findings. The operative time was 452±75.12 min with a mean intraoperative blood loss of 356±62.35 mL and a mean hospital stay of 15 ± 4.61 days in these cases. One patient had bile leakage and 3 patients had pleural effusion postoperatively, and they were discharged after drainage and medications. No liver failure or death occurred in these cases perioperatively.@*CONCLUSIONS@#3D visualization and 3D printing can facilitate accurate preoperative assessment, surgical planning and surgical procedure optimization for Bismuth-Corlette type Ⅲ and Ⅳ hilar cholangiocarcinoma to improve surgical safety and reduce surgical risks especially in cases of intrahepatic vascular variations.

Comparison study for surgical outcomes of right versus left side hemihepatectomy to treat hilar cholangiocellular carcinoma

PURPOSE: Major liver resection and radical lymph node dissection has been accepted as a definite treatment of choice for hilar cholangiocarcinoma (HC). However, the perioperative and survival outcomes of right hemihepatectomy (RH) and left hemihepatectomy (LH) still remain controversial. Thus, this study aimed to compare the surgical and oncological outcomes of RH and LH in HC patients.METHODS: From January 2000 to January 2018, a total of 326 patients underwent surgical resection for HC at Yonsei University College of Medicine in Seoul, Korea. Among the 326 patients, we excluded 130 patients and selected 196 patients, who underwent hemihepatectomy with caudate lobectomy. Among these 196 patients, 114 patients underwent RH, and 82 patients underwent LH. We compared the clinicopathological features as well as the surgical and oncologic outcomes of the RH and LH groups.RESULTS: There were no significant differences in disease-free survival (P = 0.473) or overall survival (P = 0.946) in the RH and LH groups. The LH group had fewer complications compared with the RH group, including postoperative ascites (RH: 15 [13.2%] vs. LH: 3 [3.7%], P = 0.023); however, the LH group had more bile leakage complications (RH: 5 [4.4%] vs. LH: 12 [14.6%], P = 0.012). The average time lag from portal vein embolization to operation was 25.80 ± 12.06 days (n = 45). There was no difference in postoperative liver failure (P = 0.402), although there were significantly more frequent ascites after RH (P = 0.023).CONCLUSION: LH might be a good alternative option for the surgical treatment of HC given appropriate tumor location and biliary anatomy indications.

Colangiocarcinoma. Evaluación por Tomografía Computada y Resonancia Magnética / Cholangiocarcinomas. Evaluation by Computed Tomography and Magnetic Resonance

Resumen El colangiocarcinoma (CC) es el segundo tumor primario maligno más frecuente del hígado. La mayor incidencia se registra en Asia y su pronóstico es poco alentador. La resección quirúrgica del tumor es el único tratamiento potencialmente curativo. Sin embargo, la mayoría de los CC se diagnostican en estadios avanzados de la enfermedad y la recurrencia del tumor es común. Este trabajo pretende demostrar las características imagenológicas de los CC, su clasificación, así como también la evaluación preoperatoria por tomografía computada (TC) y resonancia magnética (RM) que el radiólogo debe realizar actualmente.

Abstract The cholangiocarcinoma (CC) is the second most common malignant primary tumor of the liver. The highest incidence is registered in Asia and its prognosis is not very encouraging. The surgical resection is the only potentially curative treatment, however most of the CCs are diagnosed in advanced stages and the tumor recurrence is frequently presented. The purpose of this study is to show the imagenologic characteristics of CC, its classification as well as the preoperative evaluation by Computed Tomography (CT) and Magnetic Resonance (MR) that the radiologist must perform currently.

Relationship between serum MMP-9 level and prognosis after radical resection for Hilar cholangiocarcinoma patients

Abstract Purpose: To analyze the preoperative serum matrix metalloproteinase-9 (MMP-9) levels and prognosis of patients with hilar cholangiocarcinoma (HC) undergoing radical resection. Methods: Preoperative serum MMP-9 levels in patients with HC undergoing radical resection were detected by enzyme-linked immunosorbent assay (ELISA). The ROC curve assay was used to analyze the preoperative serum MMP-9 level to determine the most valuable cut-off point. The relationship between MMP-9 and clinicopathological features of HC patients was analyzed. Kaplan-Meier method was used to analyze the prognostic factors, and COX regression model was used to analyze the independent risk factors affecting prognosis. Results: Preoperative serum MMP-9 levels were significantly elevated in the death patients compared with the survival patients. The most valuable cut-off point for preoperative serum MMP-9 for prognosis was 201.93 ng/mL. Preoperative serum MMP-9 was associated with Bismuth-Corlette classification) and lymph node metastasis. Kaplan-Meier analysis showed that MMP-9, Bismuth-Corlette classification, Lymph node metastasis, Portal vein invasion, Hepatic artery invasion, Liver invasion, Incised margin, and Preoperative biliary drainage were related to prognosis. Cox regression model confirmed that hepatic artery invasion, liver invasion, incised margin, and MMP-9 have the potential to independence predicate prognosis in HC patients. Conclusion: Preoperative serum MMP-9 has high predictive value for prognosis and is an independent influencing factor for the prognosis of patients with hilar cholangiocarcinoma.

Expert recommendation for operational norms of laparoscopic radical resection of perihilar cholangiocarcinoma / 中华外科杂志

Radical resection is the only curable treatment for perihilar cholangiocarcinoma.With the continuous renewal of laparoscopic instruments and the continuous improvement of technology, laparoscopic radical resection of perihilar cholangiocarcinoma has been gradually carried out in China, and its feasibility and safety have been recognized by some domestic peers. In order to standardize clinical diagnosis and treatment behavior, ensure patients receive safe and standardized treatment and improve prognosis, so that the operation can be standardized application and development. Based on the principles of treatment of perihilar cholangiocarcinoma and the corresponding technical norms of laparoscopic operation, the Expert Group on Operational Norms of Laparoscopic Radical Resection of Perihilar Cholangiocarcinoma and Editorial Board of Chinese Journal of Surgery have organized relevant domestic experts to formulate expert recommendations for laparoscopic radical resection of perihilar cholangiocarcinoma, so as to facilitate clinical practice and standardized application.

Squamous Cell Carcinoma of the Extrahepatic Common Hepatic Duct

We report a rare case of hilar squamous cell carcinoma. A 62-year-old Korean woman complaining of nausea was referred to our hospital. Her biliary computed tomography revealed a 28 mm-sized protruding solid mass in the proximal common bile duct. The patient underwent left hemihepatectomy with S1 segmentectomy and segmental excision of the common bile duct. Microscopically, the tumor was a moderately differentiated squamous cell carcinoma of the extrahepatic bile duct, without any component of adenocarcinoma or metaplastic portion in the biliary epithelium. Immunohistochemically, the tumor was positive for cytokeratin (CK) 5/6, CK19, p40, and p63. Squamous cell carcinoma of the extrahepatic bile duct is rare. To date, only 24 cases of biliary squamous cell carcinomas have been reported. Here, we provide a clinicopathologic review of previously reported extrahepatic bile duct squamous cell carcinomas.

Prospective Multicenter Study of the Challenges Inherent in Using Large Cell-Type Stents for Bilateral Stent-in-Stent Placement in Patients with Inoperable Malignant Hilar Biliary Obstruction

BACKGROUND/AIMS: Although endoscopic bilateral stent-in-stent placement is challenging, many recent studies have reported promising outcomes regarding technical success and endoscopic re-intervention. This study aimed to evaluate the technical accessibility of stent-in-stent placement using large cell-type stents in patients with inoperable malignant hilar biliary obstruction. METHODS: Forty-three patients with inoperable malignant hilar biliary obstruction from four academic centers were prospectively enrolled from March 2013 to June 2015. RESULTS: Bilateral stent-in-stent placement using two large cell-type stents was successfully performed in 88.4% of the patients (38/43). In four of the five cases with technical failure, the delivery sheath of the second stent became caught in the hook-cross-type vertex of the large cell of the first stent, and subsequent attempts to pass a guidewire and stent assembly through the mesh failed. Functional success was achieved in all cases of technical success. Stent occlusion occurred in 63.2% of the patients (24/38), with a median patient survival of 300 days. The median stent patency was 198 days. The stent patency rate was 82.9%, 63.1%, and 32.1% at 3, 6, and 12 months postoperatively, respectively. Endoscopic re-intervention was performed in 14 patients, whereas 10 underwent percutaneous drainage. CONCLUSIONS: Large cell-type stents for endoscopic bilateral stent-in-stent placement had acceptable functional success and stent patency when technically successful. However, the technical difficulty associated with the entanglement of the second stent delivery sheath in the hook-cross-type vertex of the first stent may preclude large cell-type stents from being considered as a dedicated standard tool for stent-in-stent placement.

Proximal Resection Margins: More Prognostic than Distal Resection Margins in Patients Undergoing Hilar Cholangiocarcinoma Resection / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Even though the therapeutic gold standard of hilar cholangiocarcinoma (HCCA) resection is cancer-free resection margin (RM), surgical treatment still remains challenging. This study evaluated the prognostic significance of RM status in resected HCCA patients and identified survival prognostic factors. MATERIALS AND METHODS: We reviewed records of 96 HCCA patients who underwent surgery from 2001 to 2012 and analyzed the RM status and prognostic factors that affecting survival. RESULTS: Negative RM (n=31, 33%) was significantly associated with better survival vs. positive RM (n=65, 67%) (mean survival time [MST], 33 months vs. 21 months; p=0.011). Margins with histological findings of non-dysplastic epithelium, low-grade dysplasia, and carcinoma in situ were not associated with survival differences (MST, 33 months vs. 33 months vs. 30 months; p=0.452), whereas positive margins were associated with poorer survival relative to carcinoma in situ (MST, 30 months vs. 21 months; p=0.050). Among patients with R0 resection, narrow (≤ 5 mm) and wide (> 5 mm) margins were not associated with survival differences (MST, 33 months vs. 30 months; p=0.234). Although positive proximal RM was associated with poorer survival compared to negative RM (MST, 19 vs. 33; p=0.002), no survival difference was observed between positive and negative distal RMs (MST, 30 vs. 33; p=0.628). Proximal RM positivity (hazard ratio [HR], 2.688; p=0.007) and nodal involvement (HR, 3.293; p < 0.001) were independent survival prognostic factors. CONCLUSION: A clear RM, especially proximal RM status, was significant prognosticator, and proximal bile duct resection to the greatest technically feasible extent may be necessary, with careful consideration of the potential morbidity and oncologic outcomes after resection. However, an aggressive approach to obtain a negative distal RM might be controversial and should be considered carefully, depending on the patient's status.

Percutaneous Metallic Stent Placement for Palliative Management of Malignant Biliary Hilar Obstruction

OBJECTIVE: To investigate the outcomes of percutaneous metallic stent placements in patients with malignant biliary hilar obstruction (MBHO). MATERIALS AND METHODS: From January 2007 to December 2014, 415 patients (mean age, 65 years; 261 men [62.8%]) with MBHO were retrospectively studied. All the patients underwent unilateral or bilateral stenting in a T, Y, or crisscross configuration utilizing covered or uncovered stents. The clinical outcomes evaluated were technical and clinical success, complications, overall survival rates, and stent occlusion-free survival. RESULTS: A total of 784 stents were successfully placed in 415 patients. Fifty-five patients had complications. These complications included hemobilia (n = 19), cholangitis (n = 13), cholecystitis (n = 11), bilomas (n = 10), peritonitis (n = 1), and hepatic vein-biliary fistula (n = 1). Clinical success was achieved in 370 patients (89.1%). Ninety-seven patients were lost to follow-up. Stent dysfunction due to tumor ingrowth (n = 107), sludge incrustation (n = 44), and other causes (n = 3) occurred in 154 of 318 patients. The median overall survival and the stent occlusion-free survival were 212 days (95% confidence interval [CI], 186−237 days) and 141 days (95% CI, 126−156 days), respectively. The stent type and its configuration did not affect technical success, complications, successful internal drainage, overall survival, or stent occlusion-free survival. CONCLUSION: Percutaneous stent placement may be safe and effective for internal drainage in patients with MBHO. Furthermore, stent type and configuration may not significantly affect clinical outcomes.

A 2-Week Steroid Trial for Differentiating Isolated IgG4-Related Sclerosing Cholangitis from Cholangiocarcinoma / 대한췌담도학회지

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is rare disease which is steroid-responsive and often associated with IgG4 related systemic disease such as autoimmune pancreatitis. It is characterized by increased serum IgG4 and IgG4-positive lymphoplasmacytic infiltration in bile ducts. It is often difficult to distinguish IgG4-SC to hilar cholangiocarcinoma if it manifests as an isolated bile duct. We report a case of 79-year-old woman with IgG4-SC which was difficult to distinguish hilar cholangiocarcinoma due to similar clinical and radiologic findings, showing good therapeutic effect after a 2-week steroid trial.

Multidisciplinary Therapeutic Approach to Gallbladder Cancer / 대한췌담도학회지

Gallbladder cancer is the most common of all the biliary tract cancers. Incidence gradually increases with age, and women are more likely diagnosed with gallbladder cancer. Patients with gallbladder cancer have poor prognosis due to early local and vascular invasion, extensive regional lymph node metastasis, and distant metastasis. Gallbladder cancer is also related with shorter median survival duration and shorter survival duration after recurrence in comparison with hilar cholangiocarcinoma. Complete removal with negative margins is considered as the only curative therapy for patients with gallbladder cancer. The optimal resection comprises cholecystectomy with a limited hepatic resection (segments IVB and V) and portal lymphadenectomy to include the tumor with negative margins. The optimum adjuvant therapy for patients with resected gallbladder cancer has not been confirmed. The greater benefit of radiation therapy was achieved in patients with T2 or greater stage tumors and node-positive disease. Primary options for patients with unresectable or metastatic diseases include: 1) clinical trial; 2) fluoropyrimidine-based or gemcitabine-based chemotherapy; or 3) best supportive care including biliary drainage. In patients with advanced stage and jaundice, biliary drainage should be considered before setting up chemotherapy.

Which is better for unresectable malignant hilar biliary obstruction: Side-by-side versus stent-in-stent?

Biliary drainage is required for the management of unresectable malignant hilar biliary obstruction (UMHBO), and endoscopic transpapillary drainage is the first-line therapy because it is less invasive. Self-expandable metallic stents (SEMSs) are superior to plastic stents because they have longer stent patency and are more cost-effective. Endoscopic bilateral SEMS placement is technically challenging compared to unilateral placement. However, recent developments in devices and techniques have facilitated bilateral SEMS placement. There are two methods for bilateral hilar SEMS placement for UMHBO: side-by-side (SBS) and stent-in-stent (SIS). Sequential SBS was commonly conducted for bilateral hilar SEMS placement. In a new and thinner delivery system that was developed for SEM placement, two SEMSs could be simultaneously inserted and deployed through the working channel. This new bilateral stenting method enabled us to accomplish simultaneous SBS placement, which increased the success rate of SBS. Insertion of the guidewire and delivery of the second SEMS through the mesh of the first SEMS is challenging in SIS. Newly designed or modified SEMSs that are suitable for SIS have been developed to overcome this challenge, and these SEMSs have facilitated SIS. Uncovered SEMS has been commonly used for hilar SEMS placement, but covered SEMS (CSEMS) is another option for hilar SEMS placement, because CSEMS prevents tumor ingrowth and allows for removal of the stent for re-intervention. Therefore, CSEMS can be used for bilateral SEMS placement in SBS. There are many methods and kinds of SEMS available for bilateral SEMS placement. However, due to lack of evidence, there is no consensus on whether SBS or SIS is optimal for bilateral hilar SEMS placement. In this review, we compared various outcomes between SBS and SIS from previous studies, to clarify which method is better for bilateral SEMS placement for UMHBO.

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.