RESUMO
Primary neuroendocrine tumors (NETs) of the bile duct are extremely rare and represent only 0.2-2% of all gastrointestinal NETs. Within the biliary system, the main bile duct is the most affected site. A 28-year-old man with a 6-month history of intermittent jaundice, pruritus, and choluria. MRCP, PET-CT and endoscopic ultrasound were performed. A well-differentiated neuroendocrine neoplasia was diagnosed. Complete resection of the main bile duct was performed with lymphadenectomy of the hepatic pedicle with Roux-en-Y hepaticojejunostomy, without complications. The patient had an adequate evolution and nowadays he's disease-free. Primary neuroendocrine tumors of the bile duct are extremely rare. They may present clinically and radiologically similar to perihilar cholangiocarcinoma, which makes preoperative diagnosis difficult. Radical resection is indicated. Usually, they are well differentiated tumors, being the Ki-67 labeling index a reliable prognostic marker.
Los tumores neuroendocrinos (TNE) primarios de la vía biliar son extremadamente raros y representan sólo el 0.2-2% de todos los TNE gastrointestinales. Dentro del sistema biliar, la vía biliar principal es el sitio más afectado. Hombre de 28 años con cuadro de 6 meses de evolución caracterizado por ictericia intermitente, prurito y coluria. Se realizó colangiopancreatoresonancia magnética nuclear, PET-TC y ultrasonido endoscópico que concluyeron neoplasia neuroendocrina bien diferenciada. Se realizó resección completa de la vía biliar principal con linfadenectomía del pedículo hepático con hepaticoyeyunoanastomosis en Y de Roux, sin complicaciones. El paciente cursó adecuada evolución y se encuentra libre de enfermedad. Los tumores neuroendocrinos primarios de la vía biliar son extremadamente raros, presentándose clínica y radiológicamente como lesiones similares al colangiocarcinoma perihiliar lo que dificulta el diagnóstico preoperatorio. Está indicado su tratamiento quirúrgico radical. Suelen ser bien diferenciados, siendo el antígeno Ki-67 un marcador pronóstico confiable.
Assuntos
Neoplasias dos Ductos Biliares , Neoplasias do Ducto Colédoco , Tumores Neuroendócrinos , Masculino , Humanos , Adulto , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/cirurgia , Neoplasias do Ducto Colédoco/cirurgia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Neoplasias dos Ductos Biliares/cirurgia , FígadoRESUMO
An early and accurate diagnosis of biliary strictures yields optimal patient outcomes; however, endoscopic retrograde cholangiopancreatography (ERCP) with cytobrush/biopsy forceps has low sensitivity with a high number of false negatives. Various attempts to improve the accuracy of diagnosing indeterminate biliary strictures though ERCP-guided specimen acquisition have been proposed, such as with the use of fluorescence in situ hybridization, an endoscopic scraper, and the wire-grasping method, with modest to large improvements in sensitivity. Direct visualization of the biliary tree during peroral cholangioscopy has shown high sensitivity and specificity for the differentiation of neoplastic and non-neoplastic biliary lesions; however, there is no consensus on the visual characteristics of neoplastic lesions and moderate agreement between observers. Peroral cholangioscopy system (POCS)-guided specimen acquisition using forceps has shown inferior sensitivity compared to the visual characteristics; however, the specificity remains high. Optimal specimen processing with onsite evaluations and touch imprint cytology have been shown to improve the sensitivity and accurately diagnose nearly 90% of patients. In vivo evaluations of biliary strictures with probe-based confocal laser endomicroscopy have demonstrated high sensitivity with modest specificity for malignant biliary strictures. Optical computed tomography described reproductible criteria for malignancy detection in biliary strictures, increasing the sensitivity during ERCP evaluations. Differentiating benign causes from malignant causes of biliary strictures is a challenging task in clinical practice, with various concerns that still need to be addressed. Efforts should be made to define each diagnostic method's role in the evaluation of indeterminate biliary strictures.
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RESUMEN Objetivo: El objetivo del estudio fue describir las características clínico-patológicas de individuos diagnosticados de cáncer de hígado, vías biliares o vesícula. Materiales y métodos: Entre el 2006 y 2017, se diagnosticaron 89 pacientes (57% mujeres; media: 62 años) con estos canceres en dos hospitales nacionales de Lima, Perú. Resultados: Los resultados mostraron que, independientemente del tipo de cáncer, 64% de los participantes habían sido diagnosticados en estadios avanzados. La anemia fue más frecuente en los pacientes con cáncer de vías biliares e hígado y en estadios avanzados. Se observó mayor frecuencia (32%) de hipertensión arterial (HTA) en el grupo con cáncer de hígado. El análisis por edad mostró que en los pacientes mayores de 50 años la HTA fue más frecuente. Asimismo, sujetos menores de 50 años reportaron antecedentes de infecciones previas en mayor frecuencia (50%), siendo Hepatitis B (HBV) la más común. Conclusiones: Este estudio describe las características clínico-patológicas de base de una neoplasia poco estudiada en el ámbito nacional.
ABSTRACT Objective: The aim of the study was to describe the clinicopathological profile of patients diagnosed with liver, bile ducts or gallbladder cancer. Materials and methods: Between 2006 and 2017, 89 patients (57% female; mean age: 62 years-old) with these cancers were diagnosed at two national hospitals in Lima, Peru. Results: Most patients (64%) had advanced stages of disease. Anemia was more frequent in patients with bile duct and liver cancer and in advanced stages. Hypertension (HTN) was frequent among liver cancer patients (32%). The analysis by age showed that HTN was more frequent in patients over 50 years. Likewise, people under 50 years had more frequent history of previous infections (50%), Hepatitis B (HBV) being the most common. Conclusions: This study describes the baseline clinicopathological characteristics of a malignancy poorly studied in Peru.
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Acquired hemophilia A is an unusual bleeding disorder of autoimmune origin resulting in the formation of autoantibodies directed against coagulation factor VIII. These autoantibodies can act by partially or completely neutralizing the activation or function of the factor, or they can also accelerate its elimination from the circulation. The global incidence of the disease is 1.5 cases per million inhabitants per year. In nearly 50% of cases, an underlying disease that is presumed responsible to produce autoantibodies can be detected. We report a case with acquired hemophilia A, in a patient with Vater's ampulla adenocarcinoma.
La hemofilia adquirida A es un desorden hemorrágico inusual de origen autoinmune que resulta en la formación de autoanticuerpos dirigidos contra el factor VIII de la coagulación. Estos autoanticuerpos pueden actuar neutralizando parcial o completamente la activación o función del factor, o también pueden acelerar su eliminación de la circulación. La incidencia mundial de la enfermedad es de 1.5 casos por millón de habitantes por año. En cerca del 50% de los pacientes se puede detectar una enfermedad subyacente que se presume responsable de la producción de los autoanticuerpos. Se presenta el caso de un varón con hemofilia adquirida A, en contexto de adenocarcinoma de la ampolla de Vater.
Assuntos
Adenocarcinoma , Ampola Hepatopancreática , Neoplasias do Ducto Colédoco , Hemofilia A , Adenocarcinoma/complicações , Adenocarcinoma/diagnóstico , Autoanticorpos , Hemofilia A/complicações , Hemofilia A/diagnóstico , HumanosRESUMO
Resumen La hemofilia adquirida A es un desorden hemorrágico inusual de origen autoinmune que resulta en la formación de autoanticuerpos dirigidos contra el factor VIII de la coagulación. Estos autoanticuer pos pueden actuar neutralizando parcial o completamente la activación o función del factor, o también pueden acelerar su eliminación de la circulación. La incidencia mundial de la enfermedad es de 1.5 casos por millón de habitantes por año. En cerca del 50% de los pacientes se puede detectar una enfermedad subyacente que se presume responsable de la producción de los autoanticuerpos. Se presenta el caso de un varón con hemofilia adquirida A, en contexto de adenocarcinoma de la ampolla de Vater.
Abstract Acquired hemophilia A is an unusual bleeding disorder of autoimmune origin resulting in the formation of autoantibodies directed against coagulation factor VIII. These autoantibodies can act by partially or completely neutralizing the activation or function of the factor, or they can also accelerate its elimination from the circulation. The global incidence of the disease is 1.5 cases per million inhabitants per year. In nearly 50% of cases, an underlying disease that is presumed responsible to produce autoantibodies can be detected. We report a case with acquired hemophilia A, in a patient with Vater's ampulla adenocarcinoma.
Assuntos
Humanos , Ampola Hepatopancreática , Adenocarcinoma/complicações , Adenocarcinoma/diagnóstico , Neoplasias do Ducto Colédoco , Hemofilia A/complicações , Hemofilia A/diagnóstico , AutoanticorposRESUMO
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.
Assuntos
Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Neoplasias dos Ductos Biliares/patologia , Carcinoma Papilar/patologiaRESUMO
RESUMEN El tumor miofibroblástico inflamatorio es una neoplasia mesenquimal infrecuente, realizar el diagnóstico clínico así como el patológico por biopsias es un desafío. Presentamos un caso de un paciente pediátrico con tumor miofibroblástico inflamatorio localizado a nivel de las vías biliares. Se realizaron estudios de laboratorio así como imagenológicos en los cuales se planteó un diagnóstico inexacto, del mismo modo cuando se envió la muestra de la lesión para el análisis intraoperatorio a través de técnicas de congelación, el reporte microscópico también fue incorrecto. Sin embargo cuando se realizó la revisión de las láminas tras la inclusión de la lesión y complementando con estudios de inmunohistoquimica, se concluyó que la lesión correspondió a un tumor miofibroblástico inflamatorio.
ABSTRACT The inflammatory myofibroblastic tumor is an infrequent mesenchymal neoplasm, making the clinical as well as the pathological diagnosis by biopsies is a challenge. We present a case of a pediatric patient with an inflammatory myofibroblastic tumor located at the level of the bile ducts. We sent the lesion sample for intraoperative analysis through freezing techniques, the microscopic report was also incorrect. However, when the plates were reviewed after the inclusion of the lesion and supplemented by immunohistochemical studies, it was concluded that the lesion corresponded to an inflammatory myofibroblastic tumor.
Assuntos
Criança , Humanos , Masculino , Neoplasias do Sistema Biliar/patologia , Miofibroblastos/patologiaRESUMO
BACKGROUND: For palliation of malignant biliary obstruction (MBO), the gold-standard method of biliary drainage is endoscopic retrograde cholangiopancreatography (ERCP) with the placement of metallic stents. Endoscopic ultrasound (EUS)-guided drainage is an alternative that is typically reserved for cases of ERCP failure. Recently, however, there have been robust randomized clinical trials (RCTs) comparing EUS-guided drainage and ERCP as primary approaches to MBO. AIM: To compare EUS guidance and ERCP in terms of their effectiveness and safety in palliative biliary drainage for MBO. METHODS: This was a systematic review and meta-analysis, in which we searched the MEDLINE, Excerpta Medica, and Cochrane Central Register of Controlled Trials databases. Only RCTs comparing EUS and ERCP for primary drainage of MBO were eligible. All of the studies selected provided data regarding the rates of technical and clinical success, as well as the duration of the procedure, adverse events, and stent patency. We assessed the risk of biases using the Jadad score and the quality of evidence using the Grading of Recommendations Assessment, Development and Evaluation criteria. RESULTS: The database searches yielded 5920 records, from which we selected 3 RCTs involving a total of 222 patients (112 submitted to EUS and 110 submitted to ERCP). In the EUS and ERCP groups, the rate of technical success was 91.96% and 91.81%, respectively, with a risk difference (RD) of 0.00% (95%CI: -0.07, 0.07; P = 0.97; I 2 = 0%). The clinical success was 84.81% and 85.53% in the EUS and ERCP groups, respectively, with an RD of -0.01% (95%CI: -0.12, 0.10; P = 0.90; I 2 = 0%). The mean difference (MD) for the duration of the procedure was -0.12% (95%CI: -8.20, 7.97; P = 0.98; I 2 = 84%). In the EUS and ERCP groups, there were 14 and 25 adverse events, respectively, with an RD of -0.06% (95%CI: -0.23, 0.12; P = 0.54; I 2 = 77%). The MD for stent patency was 9.32% (95%CI: -4.53, 23.18; P = 0.19; I 2 = 44%). The stent dysfunction rate was significantly lower in the EUS group (MD = -0.22%; 95CI:-0.35, -0.08; P = 0.001; I 2 = 0%). CONCLUSION: EUS represents an interesting alternative to ERCP for MBO drainage, demonstrating lower stent dysfunction rates compared with ERCP. Technical and clinical success, duration, adverse events and patency rates were similar.
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Objetivo: Descrever o perfil clínico-epidemiológico de pacientes diagnosticados com câncer de vesícula e identificar o estadiamento do tumor estabelecido no momento do diagnóstico, bem como o padrão histopatológico no momento da biópsia. Métodos: Trata-se de estudo transversal e descritivo realizado em um serviço de referência em oncologia clínica, baseado em análise secundária de dados correspondentes aos prontuários de indivíduos com diagnóstico de câncer de vesícula biliar atendidos entre janeiro de 2007 e janeiro de 2017. Resultados: A distribuição por sexo foi de cinco mulheres (62,5%) e três homens (37,5%). A idade variou de 47 a 74 anos, com média de 61,1 anos e desvio padrão de ±9,03. Nenhum indivíduo era assintomático ao diagnóstico; seis (75%) apresentaram dor em hipocôndrio direito, dois (25%) perda de peso e dois outros (25%) massa palpável. Dos oito indivíduos, seis (75%) apresentavam estadiamento clínico (EC) IV ao diagnóstico. O perfil histopatológico apresentou 100% de adenocarcinoma, sendo uma amostra com áreas papilíferas, três moderadamente diferenciadas, três metastáticas e uma bem diferenciada. Conclusão: O perfil clínico-epidemiológico estabelecido foi maior prevalência de câncer de vesícula biliar em mulheres, com média de idade na sétima década. Predominou a cólica biliar como sintoma. O padrão de adenocarcinoma foi identificado em todos os indivíduos. Três quartos dos indivíduos apresentavam estágio avançado de doença. (AU)
Objective: to describe the clinical-epidemiological profile of patients diagnosed with gallbladder cancer, and to identify tumor staging established at the time of diagnosis, as well as the histopathological pattern at the biopsy. Methods: This is a cross-sectional and descriptive study, carried out in a reference service of clinical oncology, based on secondary analysis of data corresponding to the medical records of patients diagnosed with Gallbladder cancer seen between January-2007 and January-2017. Results: Gender distribution was of five women (62.5%), and three men (37.5%). Patients' ages ranged from 47-74 years, mean age of 61.1 years and standard deviation of ±9.03). No patient was asymptomatic at diagnosis, six (75%) had right hypochondrium pain, two (25%) showed weight loss, and two others (25%), palpable mass. Of the eight patients, six (75%) had EC IV staging at diagnosis. The histopathological profile showed 100% of adenocarcinoma, with one sample having papilliferous areas, three being moderately differentiated, three metastatic, and one well differentiated. Conclusion: The clinical-epidemiological profile established in this study had a higher prevalence of gallbladder cancer in women, with a mean age of the individual around the 7th decade. The most frequent symptom was biliary colic. The adenocarcinoma pattern was identified in all individuals. Three-quarters of them had advanced disease. (AU)