Urgent liver retransplantation due to transmission of intrahepatic cholangiocarcinoma by donor: the first report in the literature / Retransplante de fígado urgente devido à transmissão de colangiocarcinoma intra-hepático pelo doador: o primeiro relato da literatura

ABSTRACT BACKGROUND: Liver transplantation represents the best therapeutic modality in end-stage chronic liver disease, severe acute hepatitis, and selected cases of liver tumors. AIMS: To describe a double retransplant in a male patient diagnosed with Crohn's disease and complicated with primary sclerosing cholangitis, severe portal hypertension, and cholangiocarcinoma diagnosed in the transplanted liver. METHODS: A 48-year-old male patient diagnosed with Crohn's disease 25 years ago, complicated with primary sclerosing cholangitis and severe portal hypertension. He underwent a liver transplantation in 2018 due to secondary biliary cirrhosis. In 2021, a primary sclerosing cholangitis recurrence was diagnosed and a liver retransplantation was indicated. Recipient's hepatectomy was very difficult by reason of complex portal vein thrombosis requiring extensive thromboendovenectomy. Intraoperative ultrasound with liver doppler evaluation was performed. Two suspicious nodules were incidentally diagnosed in the donor's liver and immediately removed for anatomopathological evaluation. RESULTS: After pathological confirmation of carcinoma, probable cholangiocarcinoma, at frozen section, the patient was re-listed as national priority and a new liver transplantation was performed within 24 hours. The patient was discharged after 2 weeks. CONCLUSIONS: The screening for neoplasms in donated organs should be part of our strict daily diagnostic arsenal. Moreover, we argue that, for the benefit of an adequate diagnosis and the feasibility of a safer procedure, the adoption of imaging tests routine for the liver donor is essential, allowing a reduction of the costs and some potential risks of liver transplant procedure.

RESUMO RACIONAL: O transplante de fígado representa a melhor modalidade terapêutica na doença hepática crônica terminal, hepatite aguda grave e casos selecionados de tumores hepáticos. OBJETIVOS: Descrever um retransplante duplo em paciente do sexo masculino, diagnosticado com doença de Crohn e complicado com colangite esclerosante primária, hipertensão portal grave e colangiocarcinoma diagnosticado no fígado transplantado. MÉTODOS: Paciente do sexo masculino, 48 anos, diagnosticado com doença de Crohn há 25 anos e complicado com colangite esclerosante primária e hipertensão portal grave. Foi submetido a um transplante de fígado em 2018 devido a cirrose biliar secundária. Em 2021, foi diagnosticada recidiva de colangite esclerosante primária e indicado retransplante hepático. A hepatectomia do receptor foi de alta complexidade devido à trombose complexa da veia porta, exigindo extensa tromboendovenectomia. Foi realizada ultrassonografia intraoperatória com doppler hepático. Dois nódulos suspeitos foram diagnosticados incidentalmente no fígado do doador e imediatamente removidos para avaliação anatomopatológica. RESULTADOS: Após confirmação patológica de carcinoma, provável colangiocarcinoma, pela congelação, o paciente foi relistado como prioridade nacional, e novo transplante hepático foi realizado em 24 horas. O paciente teve alta após 2 semanas. CONCLUSÕES: O rastreamento de neoplasias em órgãos doados deve fazer parte de nosso estrito arsenal diagnóstico diário. Além disso, defendemos que, em benefício de um diagnóstico correto e da viabilidade de um procedimento mais seguro, a adoção de uma rotina de exames de imagem é essencial em doadores hepáticos, permitindo a redução dos custos e alguns riscos potenciais do procedimento de transplante hepático.

Neoplasia papilar intraductal de la vía biliar / Intraductal papillary neoplasm of the bile duct / Neoplasia papilar intraductal da via biliar

La neoplasia papilar intraductal de la vía biliar (NPIVB) es una entidad infrecuente caracterizada por el crecimiento exofítico papilar del epitelio biliar hacia la luz ductal. Previamente incluida en el grupo de tumores del mismo nombre de localización pancreática, presenta diferencias evidentes con ellos y desde 2010 se considera una entidad propia con demostrado potencial de malignización hacia colangiocarcinoma.

Papillary intraductal neoplasia of the bile duct (NPIVB) is a rare entity characterized by exophytic papillary growth of the biliary epithelium towards the ductal lumen. Previously included in the group of tumors of the same name in pancreatic location, it presents obvious differences with them and since 2010 it has been considered a separate entity with demonstrated potential for malignancy towards cholangiocarcinoma.

O neoplasma papilar intraductal da via biliar (NPIVB) é uma entidade infrequente por el creciento exofítico papilar do epitélio biliar hacia la luz ductal. Obviamente incluído no grupo de tumores do mismo nombre de localização pancreática, apresenta diferenças evidentes com ellos e desde 2010 se considerar uma entidade propia com potencial demonstrado de malignización hacia cholangiocarcinoma.

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)

Photodynamic therapy of extrahepatic cholangiocarcinoma using digital cholangioscopy / Terapia fotodinâmica em colangiocarcinoma extra-hepático utilizando colangioscopia digital

ABSTRACT Background: Cholangiocarcinoma is an aggressive neoplasm that usually requires palliative biliary drainage. Photodynamic therapy (PDT) has been described as a successful adjunct treatment to malignant biliary obstruction. Aim: To describe the use of digital cholangioscope to help provide laser light during biliary PDT session using locally developed light source. Method: Patient receives intravenous photosensitizer 24 h before the procedure. It starts with a regular duodenoscopy. After identification of the major papilla and retrograde cannulation, the digital cholangioscope is introduced into the common bile duct. Then, the cholangioscopic examination helps to identify the neoplastic stricture. Under direct visualization lighting catheter is advanced through the cholangioscope. Repositioning is recommended every centimeter to cover all strictured area. At the end of the procedure, a final cholangioscopy assesses the bile duct for the immediate result and adverse events. Result: This procedure was applied in one 82-year-old male due to obstructive jaundice in the last two months. EUS and ERCP revealed a severe dilation of the common bile duct associated with choledocholithiasis. Besides, was revealed dilation of hepatic duct up to a well-circumscribed hypoechoic solid mass measuring 1.8x2 cm compressing the common hepatic duct. The mass was deemed unresectable and the patient was referred for palliative treatment with PDT. He remained asymptomatic for three months. He perished due to complications 15 months after the PDT session. Conclusion: Digital cholangioscopy-guided biliary PDT is feasible and seems safe and effective as an adjunct modality in the palliation of extrahepatic cholangiocarcinoma.

RESUMO Racional: Colangiocarcinoma é neoplasia agressiva que geralmente exige drenagem biliar paliativa. A terapia fotodinâmica (TFD) tem sido descrita como tratamento adjunto bem-sucedido para tratar obstrução biliar maligna. Objetivo: Descrever o emprego do colangioscópio digital para ajudar a fornecer luz de laser durante sessão de TFD biliar usando fonte de luz desenvolvida localmente. Método: Paciente recebe fotossensibilizador intravenoso 24 h antes do procedimento que começa com duodenoscopia regular. Após a identificação da papila principal e da canulação retrógrada, o colangioscópio digital é introduzido no ducto biliar comum. Em seguida, o exame colangioscópico ajuda a identificar a estenose neoplásica. Sob visualização direta, o cateter de iluminação avança através do colangioscópio. Reposicionamento é feito a cada centímetro. Ao final colangioscopia avalia o ducto biliar quanto ao resultado imediato e a eventos adversos. Resultado: Este procedimento foi aplicado em um homem de 82 anos devido à icterícia obstrutiva nos últimos dois meses. EUS e CPRE revelaram dilatação grave do ducto biliar comum associada à coledocolitíase. Além disso, havia dilatação do ducto hepático até massa sólida hipoecóica bem circunscrita, medindo 1,8x2 cm, comprimindo o ducto hepático comum. Ela foi considerada irressecável e paciente encaminhado para tratamento paliativo com TFD que permaneceu assintomático por três meses. Morreu devido a complicações 15 meses após a sessão de TFD. Conclusão: A TFD biliar guiada por colangioscopia digital é viável e parece segura e eficaz como modalidade auxiliar na paliação de colangiocarcinoma extra-hepático.

Caracterización de los pacientes con tumores de la vía biliar mediante colangiopancreatografía retrógrada endoscópica / Characterization of patients with bile duct tumors by endoscopic retrograde cholangiopancreatography

Introducción: las neoplasias de la vía biliar son infrecuentes y se asocian a alta mortalidad y mal pronóstico. Ocupan el quinto lugar entre los cánceres digestivos después de estómago, colon, recto y esófago. En Cuba existe un incremento en el diagnóstico de esta entidad. Objetivo: caracterizar clínica y endoscópicamente los tumores de la vía biliar de los pacientes. Métodos: se realizó una investigación descriptiva retrospectiva en pacientes que se realizaron colangiopancreatografía retrógrada endoscópica en el Centro de Investigaciones Médico Quirúrgicas entre enero del 2006 y diciembre del 2014. Se revisaron todos los informes de este proceder y se determinaron las variables sociodemográficas (edad y sexo), datos clínicos por lo que se realiza, diagnóstico endoscópico y terapéuticas endoscópicas realizadas. Resultados: se realizaron 830 colangiopancreatografías retrógradas endoscópicas y se diagnosticó tumor de la vía biliar en 73 (8,8 por ciento) pacientes y ampulomas en 40 (4,8 por ciento), predominó el grupo etario entre 60 y 69 años (31,0 por ciento), el 57,8 por ciento del total de pacientes era del sexo femenino, el principal dato clínico que motivó la indicación del proceder fue el síndrome ictérico que estuvo presente en el 76,7 por ciento del total de pacientes y la colocación de endoprótesis fue la terapéutica endoscópica más empleada (80,2 por ciento de los casos). Conclusión: predominaron los pacientes del sexo femenino y la sexta década de la vida, el tumor al nivel del hepático común fue más frecuente, la colangiopancreatografía retrógrada endoscópica desempeñó el papel principal en el diagnóstico y tratamiento paliativo de este grupo de pacientes(AU)

Introduction: neoplasms of the biliary tract are rare and are associated with high mortality and poor prognosis. It ranks fifth among gastrointestinal cancers after stomach, colon, rectum and esophagus. In Cuba there is an increase in the diagnosis of this entity. Objective: to characterize the clinical and endoscopic bile duct tumors of patients. Methods: a retrospective descriptive study in patients was conducted endoscopic retrograde cholangiopancreatography were performed in the CIMEQ Hospital between January 2006 and December 2014. To conduct the study were reviewed all reports of endoscopic retrograde cholangiopancreatography and sociodemographic variables (age and sex), clinical data so that the procedure is performed, endoscopic diagnosis and endoscopic treatment performed type are determined. Results: 830 proceedings of endoscopic retrograde cholangiopancreatography where bile duct tumor was diagnosed in 73 (8.8 percent) patients and ampullary in 40 (4.8 percent) patients, predominant age group between 60 and 69 years to 31,0 percent were performed , the female was present in 57.8 percent of patients, as the main clinical data that motivated the indication of proceeding was the jaundice syndrome was present in 76.7 percent of patients and placement stent was the most used endoscopic therapy in 80.2 percent of cases. Conclusion: patients were predominantly female and the sixth decade of life, the level of the common tumor was more frequent liver, endoscopic retrograde cholangiopancreatography played the leading role in the diagnosis and palliative treatment of this patient group(AU)

Intraductal malignant tumors in the liver mimicking cholangiocarcinoma: Imaging features for differential diagnosis

No abstract available.

Reactive lymphoid hyperplasia of the liver

No abstract available.

Combined hepatocellular and cholangiocarcinoma: CT findings with emphasis on multiphasic helical CT.

OBJECTIVE: To describe CT findings of patients with combined hepatocellular carcinoma and cholangiocarcinoma (HCC-CC) in correlation with clinical data and histopathological results. MATERIAL AND METHOD: Ten from 31 cases whose CT study was available were reviewed retrospectively in the aspect of imaging findings, clinical data, and pathological results. RESULTS: Most of the tumors were hypodense solitary mass with gradually enhanced after contrast administration. Bile duct dilatation was observed in two cases. The overall CT findings were more similar to CC rather than HCC despite the pathological result showing predominant HCC component. Serum alpha-fetoprotein level was normal or mildly elevated while an elevated concentration of carbohydrate antigen 19-9 was observed. Hepatitis profiles showed positive to hepatitis B virus infection in four cases and hepatitis C virus infection in one case. CONCLUSION: The diagnosis of combined HCC-CC should be considered if the tumor has similar findings to CC without bile duct dilatation on cirrhotic liver and the patient has normal or low rising of the AFP level with or without elevated CA 19-9 level. In non-cirrhotic liver, the finding is non-specific.

Radiological Spectrum of Intraductal Papillary Tumors of the Bile Ducts

Papillary tumor of the bile duct is characterized by the presence of an intraductal tumor with a papillary surface comprising innumerable frondlike infoldings of proliferated columnar epithelial cells surrounding slender fibrovascular stalks. There may be multiple tumors along the bile ducts (papillomatosis or papillary carcinomatosis), which are dilated due to obstruction by a tumor per se, by sloughed tumor debris, or by excessive mucin. Radiologically, the biliary tree is diffusely dilated, either in a lobar or segmental fashion, or aneurysmally, depending on the location of the tumor, the debris, and the amount of mucin production. A tumor can be depicted by imaging as an intraductal mass with a thickened and irregular bile duct wall. Sloughed tumor debris and mucin plugs should be differentiated from bile duct stones. Cystically or aneurysmally, dilated bile ducts in mucin-hypersecreting variants (intraductal papillary mucinous tumors) should be differentiated from cystadenoma, cystadenocarcinoma and liver abscess.

Peripheral Cholangiocarcinoma / 대한간학회지

No abstract available.