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.

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.

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.

Indicações e resultados cirúrgicos em vinte e dois doentes com tumor de Klatskin / Indications and surgical treatment in twenty-two operated patients with klatskin tumor

Introdução. O colangiocarcinoma hilar ou tumor de Klatskin é neoplasia de difícil manejo e com péssimo prognóstico. Como melhor tratamento curativo, permanece a ressecção cirúrgica radical com margens livres que, em geral, envolve hepatectomia. Objetivo. Avaliar as indicações e os resultados em curto e longo prazos do tratamento cirúrgico do tumor de Klatskin em hospital de atendimento terciário. Método. Foram revisados, retrospectivamente, os prontuários de todos pacientes com diagnóstico de colangiocarcinoma hilar, operados no Hospital de Ensino da Faculdade Medicina do ABC, Santo André, São Paulo. Resultados. De setembro de 2001 a setembro de 2007, 22 pacientes foram estudados, onze homens e onze mulheres, média etária de 64 anos (de 34 a 84 anos). As lesões foram distribuídas de acordo com o sistema Bismuth-Corlette: tipo I (n = 8), tipo II (n =8) e tipo III (n= 6). Quinze doentes tiveram ressecção radical, e sete receberam tratamento cirúrgico paliativo. Na maioria dos doentes submetidos a ressecção (80%) foi realizada hepatectomia em monobloco com a via biliar. A morbidade global foi 46%, e a mortalidade 10%. A sobrevida em cinco anos foi 20% para os que tiveram a ressecção tumoral e 0% para aqueles que não tiveram ressecção. Conclusões. O tratamento cirúrgico do colangiocarcinoma hilar tem alta morbidade e significante mortalidade. A ressecção radical com hepatectomia resulta em poucos sobreviventes de longo prazo.

Introduction. Hilar cholangiocarcinoma or Klatskin tumor is a neoplasm with difficult management and dismal prognosis. The best curative treatment continues to be radical surgical resection with free margins (R0), that generally involves hepatectomy. Objective. To assess the surgical outcomes (short and long-term results) from hilar cholangiocarcinoma (Klatskin tumor) cases at a single tertiary referral center. Method.The medical records of all patients with a diagnosis of hilar cholangiocarcinoma that were submitted to surgical treatment at the Hospital de Ensino da Faculdade Medicina do ABC, Santo André, São Paulo, over a six-year period were identified and retrospectively reviewed. Results. From September 2001 through September 2007, twenty-two patients were identified. There were 11 men and 11 women. The median age was 64 years (ranged 34 to 84 years). The lesion distribution according to the Bismuth-Corlette system was: type I (n = 8), type II (n = 8), type III (n = 6). Fifteen patients underwent radical resection and seven received palliative surgical treatment. Most resected patients (80%) have main bile duct with en-bloc hepatectomy. The overall morbidity was 46%, and the mortality rate was 10%. The five-year survival rate was 20% for the patients wish resection and 0% for the non-resection cases. Conclusion. The surgical treatment of hilar cholangiocarcinoma presents high morbidity and significant mortality. Radical resection (R0) may yield a few long-term survivors.

Mulher de 69 anos de idade com obstrução biliar / A 69-year-old woman with biliary obstruction

Relata-se o caso de uma mulher com 69 anos com obstrução biliar, para enfatizar desafios no diagnóstico e no tratamento do colangiocarcinoma hilar (tumor de Klatskin). Classificado como Bismuth-Corlette tipo II, o tumor poderia ser ressecado, mas o caso foi considerado inoperável, na ocasião do diagnóstico, devido a condições clínicas. Foram realizadas drenagens biliares paliativas (percutânea e endoscópica). O óbito ocorreu por insuficiência renal e choque séptico.

A 69-year-old woman presenting with biliary stricture is reported, to emphasize diagnosis and treatment challenges about hilar cholangiocarcinoma (Klatskin tumor). Classified as Bismuth-Corlette type II, the tumor could be resectable, but the patient was considered inoperable at occasion of diagnosis, due to her clinical conditions. Palliative biliary drainages (percutaneous transhepatic and endoscopic) were performed. She died due to renal failure and septic shock.

Derivação biliodigestiva no tratamento do tumor de Klatskin / Intestinal biliary bypass in Klatskin's tumor treatment

INTRODUÇÃO: Colangiocarcinomas são formas incomuns de neoplasia gastrointestinal, cuja incidência varia de 0,01% a 0,8%. Os tumores de Klatskin são colangiocarcinomas hilares originados na bifurcação do ducto hepático principal. Atualmente a maioria dos tratamentos é paliativa. OBJETIVOS: Relatar um caso de tumor de Klatskin submetido à drenagem cirúrgica da via biliar intra-hepática. RELATO DO CASO: Homem de 68 anos, procurou assistência médica com quadro ictérico intenso e colangiorressonância magnética confirmou o diagnóstico de tumor de Klatskin. Levado à laparotomia exploradora verificou-se massa em hilo hepático com invasão portal, hepática e do complexo duodenopancreático. Optou-se por derivação biliodigestiva e hepatectomia parcial esquerda com anastomose hepaticojejunal. Houve boa evolução no pós-operatório, com diminuição da icterícia. CONCLUSÃO: A derivação biliodigestiva no tratamento do tumor de Klatskin é procedimento que alivia o quadro ictérico melhorando a qualidade de vida, e pode ser utilizado quando há irressecabilidade tumoral.

INTRODUCTION: Cholangiocarcinomas are uncommon (0,01 to 0,8%) forms of gastrointestinal neoplasms. The Klatskin tumour is ductal cholangiocarcinoma originated on the bifurcation of main hepatic duct. Currently, the majority of cases are treated in palliative way. CASE REPORT: Man with 68 years old seek medical assistance with intense jaundice and MRI gave the final diagnosis of Klatskin tumour. Exploratory laparotomy showed big hepatic mass involving portal vein and neighboring tissues. He was submitted to palliative procedure with hepatojejunal anastomosis and partial hepatectomy. He was discharged from the hospital with better clinical conditions and alleviated from his jaundice. CONCLUSION: Intestinal biliary bypass in Klatskin is an indicated procedure to alleviate the jaundice in the terminal phase of this tumour, improving the quality of live.

Traqueobroncopatia osteocondroplástica em portador de tumor de Klatskin: relato de caso e revisão da literatura / Tracheopathia osteochondroplastica in a patient with Klatskin tumor: a case report and review of the literature

A traqueopatia osteocondroplástica (TO) é uma desordem idiopática, incomum, caracterizada pela presença de nódulos osteocartilaginosos na submucosa das vias aéreas, causando rigidez e estreitamento da árvore respiratória. Afeta principalmente homens acima dos 50 anos com manifestações clínicas devidas à obstrução e/ou a infecções locais. Sua patogênese é desconhecida. Relatamos um caso de TO encontrada acidentalmente em autopsia de mulher com 73 anos de idade, que apresentava carcinoma ductal biliar extra hepático (tumor de Klatskin).

Tracheopathia osteochondroplastica (TO) is an unusual idiopathic disorder, characterized by osteocartilaginous nodules in the submucosa of the respiratory airway, which causes rigidity and narrowing of the respiratory tree. It affects mainly men over 50 and clinical manifestations are due to obstruction and/or local infections. The pathogenesis is uncertain. We report a case of TO found incidentally in an autopsy of a 73 year-old woman, who had an extrahepatic biliary ductal carcinoma (Klatskin tumor).