Hemofilia paraneoplásica adquirida secundaria a adenocarcinoma de la ampolla de Vater / Acquired paraneoplastic hemophilia secondary to Vater's ampulla adenocarcinoma

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.

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

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

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

Emprego da papilectomia endoscópica para tratamento de tumores precoces da papila duodenal maior - série de casos / Endoscopic papillectomy for early ampullary neoplastic lesions - a case series analysis

ABSTRACT BACKGROUND: Endoscopic papillectomy has been conceived as a less invasive therapeutic option for treatment of early neoplastic lesions located at the major duodenal papilla. OBJECTIVE: Evaluating patients with early ampullary lesions who underwent curative intent endoscopic papillectomy related to technical success (histopathological tumor margin assessment) and safety (adverse event rate). METHODS: A retrospective study including consecutive patients who underwent curative intent endoscopic papillectomy for demographic, histopathological and pre-/post-procedural clinical assessment. Endpoints were technical success (histopathological residual tumor assessment) and adverse events rates. RESULTS: A total of 21 medical records patients with a female predominance (13 cases, 61.9%) were included. The tumor was incidental in 8 (38%) cases. Negative residual tumor resection margin rate was 72% (15 cases); three of these cases confirmed high-grade dysplasia in the resected specimen, and six cases were invasive neoplasia. Tumoral recurrence was seen in two cases, and median follow-up time was 12 months, with a 23% loss rate (five patients). Six (28.5%) patients had adverse events, all of them early (bleeding and pancreatitis); none of them required surgical intervention and there was no mortality. CONCLUSION: Endoscopic papillectomy allowed for technical successful procedure with complete removal of ampullary neoplastic lesions in the majority of cases with acceptable adverse event rates. Recurrence rate should be carefully assessed in further studies. There was a recent increase in the number of procedures. There was also a low correlation between pre- and post-histopathological assessment regarding the presence of invasive carcinoma and adenoma with high grade dysplasia, with a predominance of superficial neoplastic adenomatous lesions.

RESUMO CONTEXTO: A papilectomia endoscópica tem sido a opção terapêutica menos invasiva no tratamento de tumores precoces que acometem a papila duodenal maior. OBJETIVO: Avaliar pacientes com tumores ampulares precoces submetidos a papilectomia endoscópica com finalidade curativa, com relação ao sucesso técnico (avaliação histopatológica da margem tumoral) e sua segurança (taxa de eventos adversos [EAs]). MÉTODOS: Foram avaliados retrospectivamente dados demográficos, exame histopatológico e evolução clínica pré e pós-procedimento de pacientes consecutivos submetidos a papilectomia endoscópica. Os desfechos avaliados foram o sucesso técnico (avaliação histopatológica de tumor residual) e taxa de EAs. RESULTADOS: Um total de 21 prontuários de pacientes com predominância feminina (13 casos, 61,9%) foi incluído no estudo. O diagnóstico tumoral foi incidental em 8 (38%) casos. A taxa de margem de ressecção negativa foi 72% (15 casos); três destas lesões confirmaram displasia de alto grau (DAG) no espécime ressecado e seis casos de neoplasia invasora. Houve recorrência tumoral em dois casos e a mediana de seguimento foi de 12 meses, com 23% de taxa de perda de seguimento (cinco casos). Seis (28,5%) pacientes apresentaram EAs, todos precoces (hemorragia e pancreatite aguda); nenhum destes necessitou de intervenção cirúrgica e não houve mortalidade. CONCLUSÃO: A papilectomia endoscópica permitiu sucesso técnico, com a completa remoção de lesões neoplásicas ampulares na maioria dos casos com taxa de EAs aceitáveis. A taxa de recorrência tumoral deve ser cuidadosamente avaliada em estudos futuros. Houve um aumento recente do número de procedimentos realizados. Também houve baixa correlação entre o diagnóstico histológico pré e pós-procedimento para a presença de adenocarcinoma invasor e adenoma com DAG, com predomínio de lesões adenomatosas superficiais.

Ictericia obstructiva por neuroma de amputación de vía biliar: Reporte de caso / Obstructive ictericia by neuroma of amputation of via biliar: Case report

Los neuromas del conducto biliar se desarrollan a partir de las fibras nerviosas simpáticas y parasimpáticas que envuelven la pared de la vía biliar. Mujer de 44 años de edad con antecedente de colecistectomía convencional seis meses previos al ingreso acude a emergencia por ictericia obstructiva de 15 días de evolución. En los estudios de imagen impresiona la presencia de una masa a nivel de las vías biliares considerándose el diagnostico de una neoplasia maligna. Por los antecedentes, ausencia de marcadores tumorales se decide realizar una biopsia percutánea sin resultados concluyente, realizándose posteriormente una intervención quirúrgica con estudio anatomo-patológico compatible con neuroma de amputación de vía biliar. El neuroma de amputación a nivel de la vía biliar es un tumor infrecuente. Puede manifestarse clínicamente como una ictericia obstructiva y suele simular a un tumor maligno de las vías biliares. El manejo quirúrgico es el tratamiento definitivo.

Neuromas of the bile duct develop from the sympathetic and parasympathetic nerve fibers that surround the wall of the bile duct. A 44-year-old woman with a history of conventional cholecystectomy six months prior to hospital admission attended emergency due to obstructive jaundice that lasted 15 days. In the imaging studies, the presence of a mass at the level of the bile ducts is considered, considering the diagnosis of a malignant neoplasm. Due to the antecedents, the absence of tumor markers, it was decided to perform a percutaneous biopsy without conclusive results, performing later a surgical intervention with anatomopathological study compatible with neuroma of biliary tract amputation. The amputation neuroma at the level of the bile duct is an infrequent tumor. It can manifest clinically as obstructive jaundice and usually simulates a malignant tumor of the bile ducts. Surgical management is the definitive treatment.

Tratamiento del ampuloma con cirugía de Whipple. Reporte del primer caso en el hospital regional de Tumbes / Ampulloma treatment with Whipple surgery. First case report in the regional hospital of Tumbes

Se presenta el primer caso de cirugía de Whipple realizada en el Hospital Regional de Tumbes para tratamiento de ampuloma con resultado exitoso. Reporte de caso de una paciente con diagnóstico de ampuloma en la que se realizó la primera cirugía de Whipple efectuada en el Hospital Regional de Tumbes en octubre del año 2014. La paciente con diagnóstico de síndrome ictérico obstructivo por ampuloma fue sometida a cirugía de Whipple, cursó el post operatorio con mejoría paulatina, no presentó patología respiratoria, ni cursó con fiebre, la paciente toleró la vía oral, la ictericia disminuyó y fue dada de alta hospitalaria a los 13 días. Cursó con fístula pancreática de gasto bajo (30 cc/día) la cual remitió a las 3 semanas. La paciente presentó mejoría clínica evidente y con resultados quirúrgicos satisfactorios hasta la fecha. La cirugía de Whipple es la técnica quirúrgica de elección en el tratamiento del ampuloma y es factible de realizarse, en casos que lo ameriten, en hospitales de frontera si se cuenta con el personal capacitado y la logística necesaria.

To report the first case of Whipple surgery performed at the Regional Hospital of Tumbes for the treatment of ampullary adenocarcinoma with successful results. A case report of a patient with diagnosis of ampullary adenocarcinoma, in whom was performed the first Whipple surgery at the Regional Hospital of Tumbes, in October, 2014. The patient diagnosed with an obstructive jaundice syndrome due to ampullary adenocarcinoma underwent Whipple surgery. She performed the postoperative period with gradual improvement, she did not present with respiratory pathology, she not have fever, the patient tolerated the oral route, the jaundice decreased and she was discharged after 13 days. She presented low-output pancreatic fistula (30 cc/day), which remitted at 3 weeks. The patient presented evident clinical improvement and satisfactory surgical results to date. Whipple surgery is the surgical technique of choice in the treatment of the ampullary adenocarcinoma and it is feasible to perform, in cases that is indicated, in bounderies hospitals if you have the trained personnel and necessary logistics.

Pre-operative biliary drainage in the periampullary neoplasia - a systematic review / Drenagem biliar pré-operatória na neoplasia periampular - uma revisão sistemática

ABSTRACT Introduction: Periampular neoplasms represent 5% of all cancers of the gastrointestinal tract with peak incidence in the 7th decade of life. The most common clinical picture is jaundice, weight loss and abdominal pain. Considering that cholestasis is related to postoperative complications, preoperative biliary drainage was developed to improve the postoperative morbidity and mortality of icteric patients with periampular neoplasias, whether resectable or not. Objective: To describe the outcome of patients with periampullary tumors undergoing preoperative biliary drainage with pancreatoduodenectomy. Method: The search was performed in the Medline/PubMed and Virtual Health Library databases by means of the combination of descriptors of the Medical Subject Headings. Inclusion criteria were clinical trials, cohorts, studies that analyze the morbidity and mortality of preoperative biliary drainage in Portuguese, English and Spanish. Exclusion criteria were studies published more than 10 years ago, experimental studies, systematic reviews and articles with WebQualis C or smaller journal in the area of ​​Medicine I or Medicine III. Of the 196 references found, 46 were obtained for reading with quality assessed through the Checklist Strengthening the Reporting of Observational Studies in Epidemiology. Eight studies were selected for review. Results: A total of 1116 patients with a sample ranging from 48 to 280 patients and a mean age of 48 to 69 years were obtained. Of the eight studies, four observed a higher rate of bleeding in drained patients; three a higher rate of positive bile culture in the intervention group; site and cavitary infection, and biliopancreatic leaks were more common in the drainage group in two studies each. The death outcome and rate of reoperation were observed in larger numbers in the control group in one study each. Conclusion: Preoperative intervention leads to a higher rate of infectious complications and bleeding.

RESUMO Introdução : Neoplasias periampulares representam 5% de todos os cânceres do trato gastrointestinal com pico de incidência na sétima década de vida. O quadro clínico mais comum é icterícia, perda de peso e dor abdominal. Considerando que a colestase está relacionada às complicações pós-cirúrgicas, a drenagem biliar pré-operatória foi desenvolvida objetivando melhorar a morbimortalidade pós-operatória de pacientes ictéricos com neoplasias periampulares, sejam elas ressecáveis ou não. Objetivo : Descrever o desfecho de pacientes com tumores periampulares submetidos à pancreatoduodenectomia com drenagem biliar pré-operatória. Método : A busca foi realizada nas bases de dados Medline/PubMed e Biblioteca Virtual em Saúde por meio da combinação de descritores do Medical Subject Headings. Os critérios de inclusão foram ensaios clínicos, coortes, estudos que analisam a morbimortalidade da drenagem biliar pré-operatória em português, inglês e espanhol. Os critérios de exclusão foram estudos publicados há mais de 10 anos, estudos experimentais, revisões sistemáticas e artigos com revista WebQualis C ou menor na área de Medicina I ou Medicina III. Das 196 referências encontradas, 46 foram obtidas para a leitura com qualidade avaliada através do checklist Strengthening the Reporting of Observational Studies in Epidemiology. Oito estudos foram selecionados para a revisão. Resultados : Obteve-se 1116 pacientes com variação de amostra de 48 a 280 pacientes e média de idade de 48 a 69 anos. Dos oito estudos, quatro observaram maior taxa de sangramento em pacientes drenados; três maior taxa de cultura de bile positiva no grupo intervenção; infecção de sítio e cavitária, além de vazamentos biliopancreáticos foram mais comuns no grupo da drenagem em dois estudos cada. O desfecho morte e a taxa de reoperação foram observados em maior quantidade no grupo controle em um estudo cada. Conclusão : A intervenção pré-operatória leva a maior taxa de complicações infecciosas e sangramentos.

Tumor de colédoco distal vs. coledocolitíase: desafio no diagnóstico pré-operatório / Distal bile duct cancer vs. choledocholithiasis: challenge in preoperative evaluation

O diagnóstico do paciente ictérico, por vezes, pode ser uma tarefa árdua. Dentre as causas principais, destacam-se a doença litiásica, como a coledocolitíase. Por outro lado, doenças neoplásicas como neoplasias peri-ampulares e da via biliar principal apresentam grande relevância, com destaque para o tumor de via colédoco. Apresentamos relato de caso de um paciente que apresentou dúvida diagnóstica nos exames pré-operatórios de imagem (US abdominal, TC multislice e Colangiorressonância). Realizada a abordagem através da exploração transcística das vias biliares para a confirmação do diagnóstico e posterior duodenopancreatectomia laparoscópica. Este relato ilustra a dificuldade em realizar o diagnóstico diferencial desta patologia. Mesmo com exames laboratoriais e de imagem, associados à anamnese e ao exame físico detalhado, nem sempre é possível confirmar o diagnóstico no pré-operatório, muitas vezes só confirmado durante o procedimento cirúrgico.

The diagnosis of jaundiced patient can sometimes be difficult task. Among the most important causes are common bile duct and its complications. By the other side, periampullary tumors and the main biliary duct tumor also have great relevance, especially cholangiocarcinoma. In this paper, we present the case report of patient that presented doubt diagnostic in preoperative in ultrasound, multislice CT and magnetic resonance. The patient underwent a laparoscopic trancystic common bile exploration, than decide to perform the laparoscopic pancreaticoduodenectomy. This report show the difficulty to make the diagnosis in pre operative evaluation. Even with laboratory and image, associate detailed case history and physical examination, not always is possible and confirm the diagnosis, many times the diagnostic is confirm during the surgical procedure.

Neurofibroma plexiforme del colédoco: una causa rara de ictericia obstructva. Reporte de caso / Plexiform Neurofibroma in the Common Bile Duct: A Rare Cause of Obstructve Jaundice. Case Report

Paciente masculino de 68 años, con ictericia obstructva quien es llevado a sala de operaciones encontrando una lesión que ocluye el 90% de la luz del tercio medio e inferior del colédoco que es reportada como un neurofbroma plexiforme del colédoco.

Male patent, 68 years old, with obstructve jaundice. Near total obstructon of common bile duct was found during laparotomy, pathology reported a plexiform neurofbroma in the common bile duct.

Microbiological analysis of bile in patients with benign and malignant biliopancreatic diseases and its consequences / Análise microbiológica da bile em pacientes com doenças biliopancreáticas benignas e malignas e suas consequências

ABSTRACT Background Bactibilia has several consequences to human health. Objetive Assessing the bile microbiology of patients with biliopancreatic diseases in order to identify bacteria and their possible infectious complications. Methods Retrospective study of 30 bile culture samples from patients with benign and malignant biliopancreatic diseases. The samples were assessed to set the bile microbiological flora and to search for its possible link with comorbidity, carcinogenesis and postoperative infectious complications. Results Thirty bile samples from patients at mean age ≈57.7 years, mostly female (n=18), were assessed. Bactibilia was found in 12 cases, mostly in patients with benign diseases (n=8), older than 50 years (n=23) and female (n=10). Adenocarcinoma of the duodenal papilla (n=9) and cholelithiasis (n=8) were the most common diseases. Escherichia coli (n=5) and Klebsiella sp (n=3) were predominantly found in patients with benign diseases; and Klebsiella sp (n=2) and Streptococcus sp (n=2) were prevalent in cancer patients. There were postoperative infectious complications in seven cases, five of them in bactibilia-associated patients (P=0.084). Conclusion Bactibilia was found in 12 samples and Escherichia coli and Klebsiella sp were most often identified in patients with benign diseases, as well as Streptococcus sp and Klebsiella sp in cancer patients. There was a trend of higher postoperative infectious complication incidence in patients with bactibilia.

RESUMO Contexto Bacteriobilia pode produzir várias consequências para a saúde humana. Objetivo Avaliar a microbiologia da bile de pacientes com doenças biliopancreáticas para identificar bactérias e possíveis consequências. Métodos Estudo retrospectivo microbiológico. Trinta amostras de bile de pacientes com doenças biliopancreáticas benignas e malignas foram avaliadas para determinar a flora microbiológica da bile e procurar alguma possível relação dessa com comorbidades, carcinogênese e complicações infecciosas pós-operatórias. Resultados As amostras de bile foram avaliadas em pacientes, com idade média ≈57,7 anos, a maioria mulheres (n=18). Evidenciou-se bacteriobilia em 12 casos, a maioria em pacientes com doenças benignas (n=8); pacientes com mais de 50 anos (n=23) e mulheres (n=10). As doenças mais comuns foram o adenocarcinoma de papila duodenal (n=9) e a colelitíase (n=8). Escherichia coli (n=5) e Klebsiella sp (n=5) foram as bactérias mais identificadas em pacientes com doenças benignas; sendo a Klebsiella sp (n=2) e o Streptococcus sp (n=2) as que predominaram nos pacientes com cânceres. As complicações pós-operatórias exclusivamente infecciosas ocorreram em sete casos, sendo em cinco desses associados à bacteriobilia (P=0,084). Conclusão Bacteriobilia foi evidenciada em 12 amostras, sendo as bactérias mais identificadas Escherichia coli e Klebsiella sp em pacientes com doenças benignas; e Streptococcus sp e Klebsiella sp nos pacientes com câncer. Existiu uma tendência a maior incidência de complicações infecciosas pós-operatórias em pacientes com bacteriobilia.

Endoscopic versus surgical treatment of ampullary adenomas: a systematic review and meta-analysis

The aim of this study is to address the outcomes of endoscopic resection compared with surgery in the treatment of ampullary adenomas. A systematic review and meta-analysis were performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. For this purpose, the Medline, Embase, Cochrane, Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS), Scopus and Cumulative Index to Nursing and Allied Health Literature (CINAHL) databases were scanned. Studies included patients with ampullary adenomas and data considering endoscopic treatment compared with surgery. The entire analysis was based on a fixed-effects model. Five retrospective cohort studies were selected (466 patients). All five studies (466 patients) had complete primary resection data available and showed a difference that favored surgical treatment (risk difference [RD] = -0.24, 95% confidence interval [CI] = -0.44 to -0.04). Primary success data were identified in all five studies as well. Analysis showed that the surgical approach outperformed endoscopic treatment for this outcome (RD = -0.37, 95% CI = -0.50 to -0.24). Recurrence data were found in all studies (466 patients), with a benefit indicated for surgical treatment (RD = 0.10, 95% CI = -0.01 to 0.19). Three studies (252 patients) presented complication data, but analysis showed no difference between the approaches for this parameter (RD = -0.15, 95% CI = -0.53 to 0.23). Considering complete primary resection, primary success and recurrence outcomes, the surgical approach achieves significantly better results. Regarding complication data, this systematic review concludes that rates are not significantly different.

FLUCTUATING JAUNDICE IN THE ADENOCARCINOMA OF THE AMPULLA OF VATER: a classic sign or an exception? / Icterícia flutuante em adenocarcinoma de ampola de Vater: um sinal clássico ou uma exceção?

Background Some authors consider the fluctuating jaundice as a classic sign of the adenocarcinoma of the ampulla of Vater. Objetive Assessing the frequency of fluctuating jaundice in their forms of its depiction in the patients with adenocarcinoma of the ampulla of Vater. Methods Observational and retrospective study, conducted through analyses of medical records from patients subjected to pancreatic cephalic resections between February 2008 and July 2013. The pathological examination of the surgical specimen was positive to adenocarcinoma of the ampulla of Vater. Concepts and differences on clinical and laboratory fluctuating jaundice were standardized. It was subdivided into type A and type B laboratory fluctuating jaundice. Results Twenty patients were selected. One of them always remained anicteric, 11 patients developed progressive jaundice, 2 of them developed clinical and laboratory fluctuating jaundice, 5 presented only laboratory fluctuating jaundice and one did not present significant variations on total serum bilirubin levels. Among the seven patients with fluctuating jaundice, two were classified as type A, one as type B and four were not classified due to lack information. Finally, progressive jaundice was the prevailing presentation form in these patients (11 cases). Conclusion This series of cases suggested that clinical fluctuating jaundice is a uncommon signal in adenocarcinoma of the ampulla of Vater. .

Contexto Alguns autores consideram a icterícia flutuante como um sinal clássico do adenocarcinoma da ampola de Vater. Objetivo Determinar a frequência de icterícia flutuante em suas formas de apresentação nos pacientes portadores de adenocarcinoma de ampola de Vater. Métodos Estudo observacional e restrospectivo, realizado a partir de análise de prontuários médicos de pacientes submetidos à ressecção cefálica pancreática entre fevereiro de 2008 e julho de 2013, cujo anatomopatológico confirmou o diagnóstico de adenocarcinoma de ampola de Vater. Conceituação e diferenciação entre icterícia flutuante clínica e laboratorial foram padronizadas. Icterícia flutuante laboratorial foi dividida em tipo A e B. Resultados Vinte pacientes foram selecionados. Um deles permaneceu sempre anictérico, 11 pacientes desenvolveram icterícia progressiva, 2 desenvolveram icterícia flutuante clínica e laboratorial, 5 apresentaram apenas icterícia flutuante laboratorial e 1 não apresentou significativa variação nos níveis séricos de bilirrubina total. Entre os sete pacientes com icterícia flutuante, dois foram classificados como tipo A, um como tipo B e quatro não foram classificados devido à falta de informações. Finalmente, icterícia progressiva foi a apresentação mais prevalente nos pacientes selecionados (11 casos). Conclusão Este artigo sugere que a icterícia flutuante clínica é um sinal incomum em pacientes portadores de adenocarcinoma de ampola de Vater. .

Pancreatoduodenectomía durante el embarazo por adenocarcinoma de ampolla de Vater y posterior resección de recurrencia ganglionar con buen resultado a corto y largo plazo / Pancreatoduodenectomy for ampullary adenocarcinoma and and re-intervention for ganglinonar recurrency

Mujer de 30 años con 13 semanas de su segunda gestación que se presentó con síntomas de anemia severa, hemorragia digestiva alta, dolor epigástrico y baja de peso. Mediante endoscopía alta se diagnosticó un adenocarcinoma bien diferenciado de ampolla de Vater. Se le practicó pancreato-duodenectomía a las 16 semanas de su embarazo sin complicaciones. Durante el seguimiento se identificó recurrencia ganglionar loco-regional 4 meses después por lo que se le realizó parto por cesárea a las 34 semanas con neonato saludable de 2500 gr. Se decidió reintervención para resección de enfermedad ganglionar la cual se realizó con éxito. Lleva 36 meses de seguimiento sin evidencia de recidiva de la enfermedad. Su hija ha tenido un desarrollo normal.

A 30 years old woman in the 13 week of her second pregnancy who had severe anemia, upper gastrointestinal bleeding and weight loss. She was given the endoscopic diagnosis of a well differentiated ampullary adenocarcinoma. She underwent a pancreato duodenectomy during the 16 week of pregnancy without complications. After 4 months of follow up we identified a ganglionar local recurrence so that´s why she underwent a cesarean in the 34 week of pregnancy. The product was a healthy 2500 gr. newborn. We decided a reoperation for the resection of the recurrence and it was carried out successfully. Currently the patient has 36 months of follow up without evidence of recurrence and her baby has a normal grow up.

Resultados actuales de la pancreatoduodenectomía para el tratamiento de los tumores periampulares y análisis de factores pronósticos de sobrevida / Results of pancreaticoduodenectomy in the treatment of periampullary tumors

Background: The diagnosis and treatment of periampullary tumors represents a challenge for current medicine. Aim: To review the results of pancreaticoduodenectomy (PDD) in the treatment of periampullary tumors and to identify risk factors that impact the long-term survival. Patients and Methods: We performed a retrospective study of patients who underwent a PDD for periampullary tumors between 1993 and 2009. We reviewed perioperative results and long term survival. We performed a multivariate analysis for long-term survival. Results: A PDD was performed in 181 patients aged 58 ± 12 years (98 females). Piloric preservation was done in 53 percent and a pancreatogastric anastomosis was used in 94 percent of cases. Morbidity was 62 percent and postoperative mortality was 5.5 percent. Pancreatic cancer was the most frequent pathological finding in 41 percent, followed by ampullary cancer in 28 percent and distal bile duct cancer in 16 percent. Median survival was 17 months, with a five years survival of 24 percent. Survival for ampullary tumors was 28 months with a five years survival of 32 percent. The median and five years survival were 14 months and 16 percent for bile duct cancer and 11 months and 14 percent for pancreatic cancer. Multivariate analysis identified tumor type (pancreas /bile duct) and lymph node dissemination as independent predictors of mortality. Conclusions: One quarter of patients experienced long term survival. Mortality predictors were tumor type and lymph node dissemination.

Ampuloma: ¿terapia endoscópica o quirúrgica? / Ampullary neoplasms: endoscopic or surgical treatment?

Ampullary neoplasms may correspond to adenoma or adenocarcinoma. The study of its staging is performed by means of computed tomography, magnetic resonance imaging and endosonography. The appropriate classification of the stages allows for a better planning of treatment. In benign tumors and small selected carcinoma, ampullectomy (endoscopic or surgical) is an alternative. In patients with ampullary neoplasms invading deep extension or showing high risk of recurrence, without evidence of metastasis, pancreatodudodenectomy is the treatment of choice. In those cases with distant metastasis, palliative treatment is indicated.

Las neoplasias ampulares pueden corresponder a adenomas o adenocarcinomas. El estudio de etapificación se realiza con tomografía computada, resonancia magnética y endosonografía. La correcta clasificación en estadíos, permite planificar de mejor forma el tratamiento. En tumores benignos y seleccionados carcinomas pequeños, la ampulectomía (endoscópica o quirúrgica) es una alternativa. En pacientes con neoplasias ampulares que presentan extensión en profundidad o alto riesgo de recurrencia, sin evidencia de metástasis a distancia, estaría indicada la pancreatoduodenectomía. En aquellos casos con metástasis a distancia, se aplican técnicas paliativas.

Estudo da linfadenectomia radical comparada à linfadenectomia standard no tratamento cirúrgico do adenocarcinoma da papila de Vater / Comparative study of the radical and standard lymphadenectomy in the surgical treatment of adenocarcinoma of the ampula of Vater

OBJETIVO: avaliar a morbidade e a mortalidade cirúrgica em pacientes submetidos a gastroduodenopancreatectomia (GDP) com linfadenectomia padrão e radical para adenocarcinoma de papila, analisando os fatores prognósticos com relação à sobrevida global e livre de doença. MÉTODOS: foram analisados retrospectivamente no período de 1999 a 2007, no Serviço de Cirurgia Abdômino-Pélvica do INCa-RJ, 50 casos de GDP para adenocarcinoma da papila duodenal divididos em dois grupos de acordo com a linfadenectomia (grupo A: linfadenectomia padrão e grupo B: linfadenectomia radical). RESULTADOS: A mediana de idade foi semelhante nos dois grupos, assim como a distribuição entre os sexos. Na comparação entre as linfadenectomias somente o número de linfonodos ressecados (grupo A: 12,3 e grupo B: 26,5) e o tempo operatório (grupo A: 421 e grupo B: 474) foram significativamente diferentes. Não ocorreram diferenças estatisticamente significativas nos dois grupos com relação a morbi-mortalidade operatória e tempo de internação. A sobrevida livre de doença (grupo A: 35 meses e grupo B: 51 meses) e sobrevida global (grupo A: 38 meses e grupo B: 53 meses) foi maior no grupo da linfadenectomia radical, porém não foram significantes estatisticamente. CONCLUSÃO: no presente estudo não ocorreram casos de linfonodos metastáticos para outros grupos nodais sem o acometimento linfonodal das cadeias pancreato-duodenais (13 e 17), sugerindo um padrão de disseminação linfonodal. Apesar da linfadenectomia radical apresentar taxas de sobrevida livre de doença e sobrevida global maiores esses dados não foram significativos estatisticamente. Novos estudos devem ser realizados para avaliar o real papel da linfadenectomia radical no adenocarcinoma da papila duodenal.

OBJECTIVE: To evaluate the morbidity and mortality in patients undergoing surgical pancreatoduodenectomy (PD) in standard and radical lymphadenectomy for adenocarcinoma of papilla, analyzing the prognostic factors related to overall and disease-free survival. METHODS: Were analyzed retrospectively from 1999 to 2007, in the Department of Abdominal and Pelvic Surgery (INCa-RJ), 50 cases of PD for adenocarcinoma of the duodenal papilla divided into two groups according to lymphadenectomy (group A: standard lymphadenectomy and group B: radical lymphadenectomy). RESULTS: The median age was similar in both groups, as well as the distribution between the sex. In the comparison between the lymphadenectomys only the number of lymph nodes resected (group A: 12.3 and group B: 26.5) and operative time (group A: 421 and group B: 474) were significantly different. There were no statistically significant differences in the two groups with respect to morbidity and mortality rate and length of hospitalization. The disease-free survival (group A: 35 months and group B: 51 months) and overall survival (group A: 38 months and group B: 53 months) was higher in the group of radical lymphadenectomy, but were not statistically significant. CONCLUSION: In this study there were no cases of metastatic lymph nodes to other groups without nodal involvement of the pancreatic-duodenal lymph node chains (13, 17), suggesting a pattern of lymph node spread. Despite the radical lymphadenectomy present rates of disease-free survival and overall survival largest such data were not statistically significant. Further studies should be conducted to evaluate the real role of radical lymphadenectomy in adenocarcinoma of the duodenal papilla.

Adenocarcinoma de la ampolla de Vater: a propósito de un caso / Vater's ampulla adenocarcinoma: a propos of a case

El 99 por ciento de los tumores malignos de la ampolla de Vater, son carcinomas. Resultan infrecuentes y de difícil diagnóstico, ya que concurren en el área, las patologías pancreáticas, del tercio distal del conducto biliar común, conducto pancreático y mucosa duodenal adyacente. El término carcinoma ampular no sólo hace referencia a una ubicación topográfica, sino también al origen histológico de los mismos, pues implica que deriva de la mucosa intestinal que tapiza dicha región. Se comunica un caso diagnosticado en el Hospital Militar Docente Dr. Mario Muñoz Monroy, de Matanzas, que acudió con un síndrome ictérico, y luego de realizada la colangiografía retrógrada endoscópica (CPRE) con toma de biopsia, ultrasonido y TAC abdominal, se pudo llegar al diagnóstico de adenocarcinoma mucoproductor de la ampolla de Vater. Fue sometido a cirugía radical con resultado exitoso...

99 per cent of the Vater's ampulla malignant tumours are carcinomas. They are infrequent and of difficult diagnosis, because there is a concurrence in the area of pancreatic diseases, of the distal third of the common bile duct, of the pancreatic duct and the adjacent duodenal mucosa diseases. The term ampullar carcinoma refers not only to a topographic location but also to their histological origin; because it implies that it derives from the intestinal mucosa that coats the region. We deal with a case diagnosed at the Teaching Military Hospital Dr. Mario Muñoz Monroy, of Matanzas, assisting the hospital with an icteric syndrome. After making an endoscopic retrograde cholangiography with biopsy, ultrasound and abdominal tomography, we arrived to the diagnosis of a Vater's ampulla mucoproductor adenocarcinoma. The patient received a radical surgery with successful results...

Hepatectomía derecha tras extirpación de tumor de la ampolla de Vater / Right hepatectomy after excision of a carcinoma of the ampulla of Vater: report of one case

Occasionally hepatectomy for metastases of ampulla of Vater carcinoma can result in a better survival and quality of life of patients. We report a 67 years old male subjected to a duodenopancreatectomy for a carcinoma of the ampulla of Vater that required afterwards a right hepatectomy for metastases. Twenty one months after the second operation and 42 months after the first operation, that patient is asymptomatic and without evidences of relapse.

El tratamiento quirúrgico del carcinoma de ampolla de Vater presenta mejores resultados oncológicos que los del resto de los tumores periampulares. En casos seleccionados, la resección hepática por metástasis de carcinoma de ampolla de Vater extirpado previamente, puede proporcionar supervivencias prolongadas y con buena calidad de vida. Presentamos un paciente de 67 años tratado con duodenopancreatectomía por cáncer de la ampolla de Vater y posteriormente con hepatectomía derecha por metástasis. A los 42 y 21 meses de la primera y segunda intervención respectivamente, permanece asintomático y sin evidencia de enfermedad.

Tumores ampulares: comunicación de un caso y revisión de la literatura / Ampullary tumors: a case report and review of the literature

Los tumores ampulares corresponden a aquellos ubicados en la unión de los segmentos terminales del conducto pancreático y colédoco. Son poco frecuentes, constituyendo entre un 1 a 2 por ciento de los tumores del tracto digestivo. Se caracterizan por su lento crecimiento y por corresponder a los tumores periampulares de mejor pronóstico. Actualmente está en discusión el enfrentamiento terapéutico, en cuanto al tipo de resección para cada tumor y cada paciente. La postura previa ha sido la resección quirúrgica clásica, no obstante está en boga un manejo conservador. Se da a conocer el caso clínico de un hombre de 53 años, que consulta por cuadro de ictericia progresiva, coluria, acolia y dolor abdominal. La ecotomografía y TC de abdomen son sugerentes de neoplasia periampular. La colangiografia endoscópica retrógrada confirmó un tumor ampular de aproximadamente 3 cm. de diámetro. Se realiza papilotomía, instalación de endoprótesis biliar y biopsia de papila. El estudio histopatológico no descarta una neoplasia invasora, por lo que se realiza ampulectomía endoscópica. La biopsia concluye adenoma túbulopapilar.