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.

Colangiocarcinoma y hepatocolangiocarcinoma combinado en pacientes con cirrosis / Cholangiocarcinoma and combined hepatocellular-cholangiocarcinoma in cirrhotic patients

Background: Cholangiocarcinoma (CCA) is a primary hepatic tumor, frequently found in patients with liver cirrhosis and biliary tract diseases. Its varieties include isolated CCA or "combined hepatocellular-cholangiocarcinoma" (cHCC-CCA). The latter is uncommon, with poorly defined diagnostic criteria and natural history. Aim: To characterize patients with cirrhosis with a pathological diagnosis of CCA and cHCC-CCA. Material and Methods: Forty-nine liver biopsies with a pathological diagnosis of CCA were reviewed. The clinical records of patients were reviewed to fetch demographic variables, etiology of cirrhosis and clinical presentation. Results: Eight of the 49 patients had cirrhosis (16% of CCA biopsies reviewed). Their median age was 64 (27-71) years and five were females. Four patients had CCA, three patients cHCC-CCA and one had a bifocal tumor. Patients in the CCA group were more commonly symptomatic. Alpha-fetoprotein and CA 19-9 levels were elevated in one of eight and four of six patients, respectively. Within 12 months from diagnosis, five of eight patients died. Conclusions: In most of these cases, the diagnosis of cHCC-CCA and CCA was made in the liver explant study without previous imaging diagnosis. This reinforces the usefulness of the histological study, in specific cases, prior to liver transplantation and emphasizes the importance of systematic explant exploration in these cases.

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.

Nuevos avances en cirugía hepática: evaluación y planificación preoperatoria (Parte 1) / New advances in liver surgery: evaluation and preoperative planning

Resumen Los avances en la cirugía hepática de los últimos años han permitido resecciones hepáticas más extensa y complejas para el tratamiento de diferentes patologías del hígado sin un aumento excesivo de la morbimortalidad perioperatoria. El desarrollo de diferentes técnicas, tecnologías y herramientas para la evaluación preoperatoria han mejorado la planificación quirúrgica con el uso por ejemplo de las tecnologías audiovisuales e impresión de modelos en 3 dimensiones (3D) de alta fidelidad. Otros avances, han permitido realizar una mejor evaluación funcional del parénquima hepático y una caracterización más precisa de las lesiones con el uso por ejemplo de verde de indocianina, cintigrafía hepática y resonancia magnética con contraste hepatoespecífico. Este artículo describe algunos de los nuevos avances en la evaluación y planificación preoperatoria en cirugía hepática.

Advances in liver surgery in recent years have made it possible to achieve more extensive and complex liver resections for the treatment of different liver diseases without an excessive increase in perioperative morbidity and mortality. The development of different techniques, technologies and tools for preoperative evaluation have improved surgical planning with the use, for example, of audiovisual technologies and printing of high-fidelity 3-dimensional (3D) models. Other advances have allowed a better functional evaluation of the liver parenchyma and a more precise characterization of the lesions with the use, for example of indocyanine green or liver scintigraphy and magnetic resonance with hepatospecific contrast. This article describes some of the new advances in preoperative evaluation and planning in liver surgery.

Aneurisma de la aorta abdominal asociado a íctero / Aneurysm of the abdominal aorta associated with icterus

El aneurisma de la aorta abdominal como causa de íctero obstructivo es una situación de excepción en la clínica. El objetivo de esta presentación fue exponer la coexistencia del aneurisma de la aorta abdominal y el íctero. Una asociación muy poco frecuente y de difícil diagnóstico, que coloca al cirujano vascular en la toma de una especial conducta terapéutica. La cuidadosa revisión de los antecedentes patológicos y el análisis del comportamiento clínico del paciente permitieron la solución terapéutica adecuada. Se logró demostrar que el íctero obedecía a una lesión maligna de vías biliares conocida como colangiocarcinoma, que concomitó con la existencia de un aneurisma del V segmento aórtico y no fue el resultado de una compresión extrínseca de la dilatación de la aorta, sobre las vías biliares. La conducta quirúrgica, encaminada a erradicar el íctero y evitar la ruptura aneurismática con resección del aneurisma y colocación de prótesis, no resultó posible por lo avanzado de la lesión tumoral y el grado de metástasis ya establecidos. Solo se realizó resección de la vesícula biliar y seguimiento posterior por oncología(AU)

Abdominal aortic aneurysm as a cause of obstructive icterus is an exceptional situation in the clinic. The aim of this presentation was to expose the coexistence of abdominal aortic aneurysm and icterus. A very rare and difficult to diagnose association, which places the vascular surgeon in a special therapeutic approach. The careful review of the pathological history and the analysis of the patient's clinical behavior allowed the appropriate therapeutic solution. It was possible to demonstrate that the icterus was due to a malignant lesion of the biliary tract known as cholangiocarcinoma, which concomitated with the existence of an aneurysm of the V aortic segment and was not the result of extrinsic compression of the dilatation of the aorta on the biliary tract. The surgical procedure, aimed at eradicating the icterus and avoiding aneurysmal rupture with aneurysm resection and prosthesis placement, was not possible due to the advanced tumor lesion and the degree of metastasis already established. Only gallbladder resection was performed and subsequent follow-up by oncology(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)

Intraductal papillary neoplasia of the bile duct (IPNB): case report and literature review

ABSTRACT Intraductal papillary neoplasm of the bile duct (IPNB) is a rare premalignant condition, defined as an epithelial neoplasm of the bile duct with exophytic papillary growth that can develop in any segment of the biliary tree. This pathology, with the highest prevalence in Asia, has been associated with the presence of hepatolithiasis and eastern liver infection (clonorchiasis). The diagnosis will depend on the clinical suspicion against the findings evidenced in the different diagnostic supports (invasive and non-invasive). Curative resection with negative margins is the treatment of choice in patients candidates for surgical management. In the event that the patient is not a candidate for curative resection, palliative treatment includes chemotherapy, percutaneous and endoscopic drainage, laser cholangioscopy ablation and intraluminal therapy with iridium 192. We present two cases of two patients with a diagnosis of IPNB confirmed by histology treated at our institution. The first case in an 86-year- old patient had a history of recurrent obstructive biliary syndrome and clinical suspicion of a new episode of cholangitis, and the second case in a 73-year-old patient who had a disseminated infectious process (spondylodiscitis, pelvic and intra-abdominal abscesses), and with the clinical suspicion of presenting a primary hepatobiliary focus. Both patients underwent single-operator cholangioscopy plus biopsy, confirming the diagnosis by histology. The first case was managed with palliative intent, indicating endoscopic diversion of the bile duct, while surgical management was indicated in the second case.

RESUMEN La neoplasia papilar intraductal de la vía biliar (NPIB) es una enfermedad premaligna poco frecuente, definida como una neoplasia epitelial de la vía biliar con crecimiento papilar exofítico que puede desarrollarse en cualquier segmento de la vía biliar. Esta patología, de mayor prevalencia en Asia, se ha asociado a la presencia de hepatolitiasis e infección hepática oriental (clonorquiasis). El diagnóstico dependerá de la sospecha clínica frente a los hallazgos evidenciados en los diferentes soportes diagnósticos (invasivos y no invasivos). La resección curativa con márgenes negativos es el tratamiento de elección en pacientes candidatos a manejo quirúrgico. En el caso de que el paciente no sea candidato a resección curativa, el tratamiento paliativo incluye quimioterapia, drenaje percutáneo y endoscópico, ablación por colangioscopia láser y terapia intraluminal con iridio 192. Presentamos dos casos de dos pacientes con diagnóstico de NPIB confirmado por histología tratado en nuestra institución. El primer caso de un paciente de 86 años con antecedentes de síndrome biliar obstructivo recurrente y cursando con un nuevo episodio y sospecha de colangitis aguda, y el segundo caso en un paciente de 73 años que presentaba un proceso infeccioso diseminado (espondilodiscitis, abscesos pélvicos e intraabdominales), con sospecha clínica de presentar un foco hepatobiliar primario. Ambos pacientes fueron sometidos a colangioscopia más biopsia, confirmando el diagnóstico por histología. El primer caso se manejó con intención paliativa, indicando derivación endoscópica de la vía biliar, mientras que en el segundo se indicó manejo quirúrgico.

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.

Pathological and immunohistochemical aspects of primary hepatobiliary neoplasms in cats / Aspectos patológicos e imuno-histoquímicos de neoplasias hepatobiliares primárias em gatos

Primary hepatobiliary neoplasms (PHN) are uncommon in cats, and originate in hepatocytes, intra- and extrahepatic bile ducts, mesenchymal cells, and cells of neuroendocrine origin. The aim of this study was to determine the frequency of PHN in cats diagnosed in the metropolitan region of Porto Alegre (RS), Brazil, for a period of 17 years, determining their epidemiological, anatomopathological and immunohistochemical aspects. Necropsy reports of 2.090 cats were analyzed, 125 were diagnosed with primary hepatobiliary diseases, of which 15 were cases of PHN, representing 12% of the specific hepatobiliary conditions and 0.7% of the necropsies. All PHN were malignant, of which 93.3% had epithelial origin and 6.7% presented mesenchymal origin. Cholangiocarcinoma was the most commonly diagnosed neoplasm, followed by hepatocellular carcinoma and hemangiosarcoma. In general, cats with no defined breed were the most affected. Concerning sex, 60% were females and 40% males. Age ranged from five to 18 years, with a mean age of 10.5 years (median of ten years). Grossly, cholangiocarcinoma and hemangiosarcoma were multinodular and hepatocellular carcinoma was massive. Microscopically, cholangiocarcinomas were arranged in acini and ducts, whereas hepatocellular carcinomas were arranged in solid sheets or trabeculae. On immunohistochemistry, cholangiocarcinomas, hepatocellular carcinomas, and hemangiosarcomas were positive for the antibodies CK 7, Hep Par-1, and vimentin and von Willebrand factor, respectively.(AU)

Neoplasias hepatobiliares primárias (NHP) são incomuns em gatos e se originam de hepatócitos, células dos ductos biliares intra e extra-hepáticos, células mesenquimais e ainda células de origem neuroendócrina. O objetivo do trabalho foi determinar a frequência das NHP em gatos diagnosticados na Região Metropolitana de Porto Alegre, no período de 17 anos, abordando seus aspectos epidemiológicos, anatomopatológicos e imuno-histoquímicos (IHQ). Foram analisados os laudos de necropsia de 2.090 gatos sendo que 125 foram diagnosticados com doenças hepatobiliares primárias, destes 15 foram casos de NHP, representando 12% das condições hepatobiliares específicas e 0,7% do total de necropsias. Todos os diagnósticos de NHP eram malignos, destes 93,3% apresentaram origem epitelial e 6,7% mesenquimal. Colangiocarcinoma foi a neoplasia mais diagnosticada, seguido do carcinoma hepatocelular e hemangiossarcoma. De uma maneira geral, os gatos sem raça definida foram os mais acometidos. Em relação ao sexo 60% eram fêmeas e 40% machos. A idade variou de cinco a 18 anos, com a idade média de 10,5 anos (mediana de 10 anos). Macroscopicamente o colangiocarcinoma e hemangiossarcoma eram multinodulares, e o carcinoma hepatocelular, maciço. À histologia, houve predomínio do arranjo acinar e ductal nos colangiocarcinomas e sólido, no carcinoma hepatocelular. Na IHQ os colangiocarcinomas foram reativos para CK 7, carcinoma hepatocelular para Hep Par-1 e hemangiossarcoma para vimentina e fator de von Willebrand.(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.

Transarterial radioembolization for liver tumors as neoadjuvant therapy: three case reports / Radioembolização transarterial hepática como terapia neoadjuvante: relato de três casos

ABSTRACT Transarterial radioembolization (TARE) with yttrium-90 microspheres is a palliative locoregional treatment, minimally invasive for liver tumors. The neoadjuvant aim of this treatment is still controversial, however, selected cases with lesions initially considered unresectable have been enframed as candidates for curative therapy after hepatic transarterial radioembolization. We report three cases in which the hepatic transarterial radioembolization was used as neoadjuvant therapy in an effective way, allowing posterior potentially curative therapies.

RESUMO A radioembolização transarterial hepática com microesferas de ítrio-90 é uma modalidade paliativa de tratamento locorregional minimamente invasiva. O objetivo neoadjuvante deste tratamento ainda é controverso, mas casos selecionados de lesões consideradas inicialmente irressecáveis reenquadram-se como candidatos à terapia curativa após a radioembolização transarterial hepática. Relatamos três casos em que a radioembolização transarterial hepática foi utilizada como terapia neoadjuvante de forma efetiva possibilitando aplicação posterior de terapias potencialmente curativas.

Colangiocarcinoma intrahepatico con manifestaciones clínicas atípicas: reporte de caso / Intrahepatic cholangiocarcinoma with atípical clinical manifestations, case report

El colangiocarcinoma es una neoplasia de la vía biliar, siendo el segundo cáncer hepático primario más frecuente en el mundo, con una incidencia del 2%. Predomina enhombres, la sobrevida en estados tempranos es de 40 meses con cirugíay 12 meses con quimioterapiaen estados mas avanzados.Objetivo:identificar manifestaciones clínicas no específicas, que orienten al médico a realizar un diagnóstico temprano del Colangiocarcinoma. Presentación del caso clínico: paciente masculino, en la sexta década de la vida, diabético, con historia de 18 meses de presentar fatiga y tos seca sin predominio de horario, durante el proceso diagnóstico se encontró hepatomegalia y anemia microcítica hipocrómica.La tomografía computarizada abdominal, reveló masa heterogénea en hígado. El resultado de los marcadores tumorales CA 19.9 y antígeno carcinoembrionario fueron normales.Conclusión: el colangiocarcinoma es un tipo de cáncer que se puede presentar con síntomas inespecíficos por lo que la sospecha clínica puede ser difícil y debe apoyarse en estudios complementarios...(AU)

Ureteropelvic junction obstruction caused by metastatic cholangiocarcinoma

ABSTRACT We describe the rare case of a 61-year-old female with right ureteropelvic junction (UPJ) obstruction caused by metastatic cholangiocarcinoma. Her past medical history was notable for cholangiocarcinoma treated with neoadjuvant chemoradiation and two orthotopic liver transplants six years earlier. Urology was consulted when she presented with flank pain and urinary tract infection. Diagnostic workup demonstrated right UPJ obstruction. She was managed acutely with percutaneous nephrostomy. She subsequently underwent robotic pyeloplasty and intrinsic obstruction of the UPJ was discovered. Histological examination revealed adenocarcinoma, consistent with systemic recurrence of the patient's known cholangiocarcinoma.

Seguridad y eficacia del uso de prótesis metálicas autoexpandibles en el manejo de estenosis malignas de la vía biliar irresecables / Safety and efficacy of the use of self-expanding metal stents in the management of unresectable malignant biliary stenosis

Antecedentes: En los últimos años se ha producido un incremento del uso de prótesis metálicas autoexpandibles (SEMS) en pacientes con estenosis malignas irresecables de la vía biliar. Sin embargo, en Perú no se cuentan con reportes sobre la seguridad y eficacia de este procedimiento. Objetivo: Evaluar la seguridad y eficacia del uso de las SEMS en el manejo paliativo de las estenosis malignas de la vía biliar. Materiales y métodos: Cohorte retrospectiva. Se incluyeron a todos los pacientes referidos para colocación de SEMS biliar como parte de un tratamiento paliativo entre enero del 2016 y agosto del 2018. Se obtuvieron las tasas de colocación exitosa de las SEMS, de paliación adecuada de la obstrucción y de complicaciones asociadas al procedimiento. Se evaluó la patencia de la prótesis durante el seguimiento. Se determinó la supervivencia luego de la colocación de la prótesis. Resultados: Se incluyeron 32 pacientes con indicación de manejo paliativo debido a una estenosis maligna irresecable de la vía biliar. El cáncer de páncreas (56,25%) seguido del colangiocarcinoma (31,25%) fueron las etiologías más frecuentes. Se alcanzó una tasa de colocación exitosa en primera intención de 96,97%. La paliación adecuada de la obstrucción biliar se alcanzó en el 100% de los pacientes (p<0,05). Dos SEMStc migraron durante el seguimiento (6,25%) siendo manejados con la colocación de una nueva SEMSnc. Conclusiones: La colocación de SEMS constituye una estrategia segura, con alta tasa de éxito terapéutico en el manejo paliativo de los pacientes con obstrucción maligna de la vía biliar.

Background: In recent years there has been an increase in the use of self-expanding metallic stents (SEMS) in patients with unresectable malignant stenosis of the bile duct. However, in Peru there are no reports on the safety and efficacy of this procedure. Objective: To evaluate the safety and efficacy of the use of SEMS in the palliative management of malignant stenosis of the bile duct. Materials and methods: Retrospective cohort. All patients referred for placement of biliary SEMS as part of a palliative treatment between January 2016 and August 2018 were included. Rates of successful placement of SEMS, adequate palliation of the obstruction, and complications associated with the procedure were obtained. The patency of the prosthesis was evaluated during follow-up. Survival was determined after placement of the prosthesis. Results: We included 32 patients with indication of palliative management due to an unresectable malignant stenosis of the bile duct. Pancreatic cancer (56.25%) followed by cholangiocarcinoma (31.25%) were the most frequent etiologies. A successful first-time placement rate of 96.97% was achieved. Adequate palliation of biliary obstruction was achieved in 100% of patients (p<0.05). Two SEMStc migrated during follow-up (6.25%) being managed with the placement of a new SEMSuc. Conclusions: The placement of SEMS is a safe and effective therapeutic strategy in the palliative management of patients with malignant obstruction of the biliary tract.

Fasciolosis intrahepática que simulaba un colangiocarcinoma: Reporte de un caso infrecuente / Intrahepatic fasciolosis that simulated a cholangiocarcinoma: Report an infrequent case

La fasciolosis es una infección parasitaria causada por 2 tipos de fasciola, siendo la más común de tipo hepática, la forma infectante son las metacercarias. Su prevalencia radica en zonas de nivel socioeconómicas bajo y zonas ganaderas. El objetivo es presentar un caso de presentación atípica en el que se evitó someter al paciente a un tratamiento quirúrgico innecesario. Presentamos un caso de un paciente masculino de 69 años que presentó Colangitis, su ecografía y tomografía evidenció dilatación de la vía biliar, por lo que se le realizó una con la colangiopancreatografía retrograda endoscópica (CPRE) evidenciándose una estenosis de colédoco cerca de la bifurcación biliar y una larva de fasciola hepática, la endomicroscopía confocal (EM) no era concluyente, se tomó biopsia de la estenosis que reportó colangiocarcinoma, pero como el paciente no tenía pérdida patológica de peso y marcadores tumorales negativos, se le indicó tratamiento antiparasitario y se realizó una nueva CPRE que evidenció en la EM mucosa inflamatoria de aspecto crónico no tumoral que se confirmó con la nueva biopsia, el paciente tuvo una evolución favorable, de esta forma evitamos un tratamiento no requerido.

Fasciolosis is a parasitic infection caused by 2 types of fasciola, being the most common of hepatic type, the infective form is the metacercariae. Its prevalence lies in low socio-economic zones and livestock areas. The objective is to present a case of atypical presentation in which the patient was avoided to undergo an unnecessary surgical treatment. We present a case of a 69-year-old male patient who presented Cholangitis, his sonography and tomography evidenced dilatation of the bile duct, and one was performed with endoscopic retrograde cholangiopancreatography (ERCP), showing a choledochal stenosis near the bile bifurcation And a liver fasciola larva, confocal endomicroscopy (MS) was inconclusive, biopsy of the stenosis that reported cholangiocarcinoma was reported, but since the patient had no pathological weight loss and negative tumor markers, he was given antiparasitic treatment and was performed A new ERCP that showed in the inflammatory mucosa of chronic non-tumor aspect that was confirmed with the new biopsy, the patient had a favorable evolution, thus avoiding an unnecessary treatment.

Gene expression profiling reveals upregulated FUT1 and MYBPC1 in children with pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is associated with high risk of epithelial atypical growth and malignant transformation of the bile duct or gallbladder. However, overall changes in genetic expression have not been examined in children with PBM. Genome-wide expression was analyzed using peripheral blood samples from 10 children with PBM and 15 pediatric controls. Differentially expressed genes (DEGs) were identified using microarray. Bioinformatics analysis was conducted using Gene Ontology and KEGG analyses. The top 5 in the up-regulated genes in PBM were verified with qRT-PCR. Receiver operator characteristic curve analysis was conducted to evaluate the predictive accuracy of selected genes for PBM. The microarray experiments identified a total of 876 DEGs in PBM, among which 530 were up-regulated and the remaining 346 were down-regulated. Verification of the top 5 up-regulated genes (TYMS, MYBPC1, FUT1, XAGE2, and GREB1L) by qRT-PCR confirmed the up-regulation of MYBPC1 and FUT1. Receiver operating characteristic curve analysis suggested that FUT1 and MYBPC1 up-regulation could be used to predict PBM, with the area under the curve of 0.873 (95%CI=0.735−1.000) and 0.960 (95%CI=0.891−1.000), respectively. FUT1 and MYBPC1 were up-regulated in children with PBM, and could be used as potential biomarkers for PBM.

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.