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.

Characteristics of benign and malignant lesions of ampullary polyps and the accuracy of forceps biopsy / 北京大学学报(医学版)

OBJECTIVE@#To distinguish the endoscopic and clinical features of ampullary polyps, to investigate the endoscopic cancer risk factors of ampullary polyps based on the compared differences of benign lesions and adenocarcinoma, and to assess the accuracy of forceps biopsy.@*METHODS@#Authors retrospectively analyzed the data extracted from patients treated with endoscopic papillectomy (EP) from January 2009 to May 2019 in the Department of Gastroenterology, Peking University Third Hospital. Endoscopic pictures and pathology reports were reevaluated and analyzed. Differences between benign and cancer groups were conducted.@*RESULTS@#In the study, 42 cases were involved, 35 to 83 years old, containing 83.3% older than 50 years old patients. The histological types were as follows, 2 for inflammatory polyps (4.8%), 1 for neuroendocrine tumor (2.4%), 1 for hyperplastic polyp (2.4%), 5 for grade Ⅰ adenoma (11.9%), 10 for grade Ⅱ adenoma (23.8%), 4 for grade Ⅲ adenoma (9.5%) and 19 for adenocarcinoma (45.2%), and 90.5% were adenoma or adenocarcinoma. The average age of benign group (inflammatory polyps and adenomas) was (56.7±9.2), which was significantly younger than that of adenocarcinoma group [(66.0±9.8), P=0.004]. Tumor diameter in adenocarcinoma group[(2.3±0.8) cm] was significantly larger than that in benign group[(1.6±0.6) cm, P=0.002]. Benign lesions only showed Yamada type Ⅰ(57.1%)and type Ⅱ(42.9%). The percentage of Yamada type Ⅰ (36.8%)and type Ⅱ(31.6%) in adenocarcinoma group was lower than that in benign group. Moreover, Yamada type Ⅲ (31.5%) was only found in the adenocarcinoma group. Significant differences were observed between the two groups in Yamada types (P=0.046). Most of the benign lesions had clear boundary(18/21, 85.7%). The percentage of clear boundary in adenocarcinoma group (2/19, 10.5%) was significantly lower than that in the benign group (P < 0.001). No significant differences were investigated in color (P=0.353) and surface (P=0.324) between benign and adenocarcinoma lesions. Pooling age, lesion diameter, Yamada type and clear boundary into Logistic regression analysis, only age (OR=1.186, 95%CI 1.025-1.373, P=0.022) and clear boundary (OR=66.218, 95%CI 3.421-1 281.840, P=0.006) were the independent cancer risk factors. Only 2 (10.5%) in the 19 cancer patients had positive biopsy results before EP. As compared with post-EP, 55.3% (21/38) biopsies were under-estimated, including 17 (17/19, 89.5%) adenocarcinomas and 4 (4/10, 40%) grade Ⅱ adenomas.@*CONCLUSION@#adenoma and adenocarcinoma were the major histological type of ampullary po-lyps. Age and unclear boundary were the independent risk factors of ampullary adenocarcinoma. Forceps biopsy was not enough for ampullary polyp differentiation.

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.

Bile duct segmental resection versus pancreatoduodenectomy for middle and distal common bile duct cancer

PURPOSE: To compare survival outcomes between bile duct segmental resection (BDR) and pancreatoduodenectomy (PD) for the treatment of middle and distal bile duct cancer. METHODS: From 1997 to 2013, a total of 96 patients who underwent curative intent surgery for middle and distal bile duct cancer were identified. The patients were divided into 2 groups based on the type of operation; 20 patients were included in the BDR group and 76 patients were in the PD group. We retrospectively reviewed the clinical outcomes. RESULTS: The number of lymph nodes (LNs) was significantly greater in patients within the PD group compared to the BDR group. The total number of LNs was 6.5 ± 8.2 vs. 11.2 ± 8.2 (P = 0.017) and the number of metastatic LNs was 0.4 ± 0.9 vs. 1.0 ± 1.5 (P = 0.021), respectively. After a median follow-up period of 24 months (range, 4–169 months), the recurrence-free survival of the PD group was superior to that of the BDR group (P = 0.035). In the patients with LN metastases, the patients undergoing PD had significantly better survival than the BDR group (P < 0.001). CONCLUSION: Surgeons should be cautious in deciding to perform BDR for middle and distal common bile duct cancer. PD is recommended if LN metastases are suspected.

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.

A Double-Layered Covered Biliary Metal Stent for the Management of Unresectable Malignant Biliary Obstruction: A Multicenter Feasibility Study

BACKGROUND/AIMS: The covered self-expandable metal stent (CMS) was developed to prevent tumor ingrowth-induced stent occlusion during the treatment of malignant biliary obstruction. However, complications such as cholecystitis, pancreatitis, and stent migration can occur after the endoscopic insertion of CMSs. The aim of the present study was to assess the efficacy and safety of a double-layered CMS (DCMS) for the management of malignant bile duct obstruction. METHODS: DCMSs were endoscopically introduced into 59 patients with unresectable malignant extrahepatic biliary obstruction at four tertiary referral centers, and the patient medical records were retrospectively reviewed. RESULTS: Both the technical and functional success rates were 100%. Procedure-related complications including pancreatitis, cholangitis, stent migration, and liver abscess occurred in five patients (8.5%). The median follow-up period was 265 days (range, 31 to 752 days). Cumulative stent patency rates were 68.2% and 40.8% at 6 and 12 months, respectively. At the final follow-up, the rate of stent occlusion was 33.9% (20/59), and the median stent patency period was 276 days (range, 2 to 706 days). CONCLUSIONS: The clinical outcomes of DCMSs were comparable to the outcomes previously reported for CMSs with respect to stent patency period and complication rates.

A Case of Adenomyomatous Hyperplasia of the Distal Common Bile Duct Mimicking Malignant Stricture / 대한소화기학회지

Adenomyomatous hyperplasia is a reactive malformation or non-neoplastic tumor-like lesion frequently observed in the gallbladder, stomach, duodenum and jejunum, but rare in the extrahepatic bile duct. A 42-year-old man with epigastric discomfort had a stricture in the common bile duct on initial CT scans. Initially, it was regarded as a malignant lesion with some evidence, but histopathologic examinations of multiple biopsies obtained by multiple sessions of endoscopic retrograde cholangiopancreatography showed no evidence of malignancy. The patient had undergone the pylorus preserving pancreaticoduodenectomy because of the possibility of malignancy; however, the final diagnosis was adenomyomatous hyperplasia. It is important to distinguish a malignancy from benign biliary stricture with endoscopic biopsies. Surgery for suspected biliary malignancy often reveals benign lesions. Therefore, a correct diagnosis is important before deciding upon treatment of bile duct stricture. In conclusion, in younger patients with bile duct stricture where there is no evidence of histologic malignancy despite multiple biopsies, the possibility of benign disease such as adenomyomatous hyperplasia should be considered, to avoid unnecessary radical surgery.

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. .

Management of patients with ampullary and periampullary tumors in gastro-entrology and hepatology teaching hospital

Background: Ampullary and periampullary tumors are composed of a group of malignant and benign neoplasms arising in the region of the ampulla of Vater and are a major problem to the patients and surgeons and their management remains a big challenge

Objective: Study the age, sex distribution, presentation and management of patients with ampullary and periampullary tumors in Iraq and compare it with other world reports

Patients and Methods: prospective study of 50 patients with ampullary and periampullary tumors carried out in GIT hospital [tertiary center] from January 2013 to March 2015, risk factors, age, sex, clinical presentation, images study, diagnosis and modality of treatments whether curative or palliative are studied

Results: 29 [58%] were males and 21[42%] were females, about the age distribution the mean age was 45 ,about the residency they were mainly from Baghdad in [40%] the presenting symptom was obstructive jaundice in 43 [86%] ,duration of illness [range from 1-2 months] with the mean period 45 days, abdominal ultrasound study reveal distended Gallbladders in 76%, dilated CBD in 82%, dilated pancreatic duct in 56%, Hepatomegaly in 42%, dilated intrahepatic billiary tree in 64%, and liver secondary in 10%, resection surgery was done in 50%, palliative surgery in 12% and endoscopic palliation in 38%

Conclusion: Ampullary and periampullary tumors are relatively common. The mean age is 45 years, lower than the western studies. Sex: male are affected 1.4 times more than female with male: female ratio = 1.38 Abdominal ultrasound is very informative but EUS, CT scan and MRI, MRCP is the cornerstone for the diagnosis, Normal looking papilla does not rule out occult ampullary tumor

Ampullary Adenoma Treated by Endoscopic Double-Snare Retracting Papillectomy

We report herein improved methods for the safe and successful completion of endoscopic papillectomy (EP). Between January 2008 and November 2011, 12 patients underwent double-snare retracting papillectomy for the treatment of lesions of the major duodenal papilla. The main outcomes were en bloc resection rates, pathological findings, and adverse events. All of the patients (mean age, 60.1 years; range, 38 to 80 years) were diagnosed with ampullary adenoma by endoscopic forceps biopsies prior to endoscopic snare papillectomy. En bloc resection by double-snare retracting papillectomy was successfully performed for all lesions (median size, 12.3 mm), comprising six tubular adenomas, one tubulovillous adenoma, three cases of epithelial atypia, one hamartomatous polyp, and one case of duodenitis with regenerative change. Significant hemorrhage and pancreatitis were observed in one case after EP. Adenoma recurrence occurred in three patients during follow-up (median, 28.5 months) at a mean interval of 2 months postoperatively (range, 1 to 3 months). No serious adverse events were observed. Double-snare retracting papillectomy is effective and feasible for treating lesions of the major duodenal papilla. Further treatment experience, including a single-arm phase II study, needs to be accumulated before conducting a randomized controlled study.

Value of PUSSOM and P-POSSUM for the prediction of surgical operative risk in patients undergoing pancreaticoduodenectomy for periampullary tumors / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the value of Physiological and Operative Severity Score for the enUmeration of Mortality and morbidity (POSSUM) and a modification of the POSSUM system (P-P0SSUM) scoring system in predicting the surgical operative risk of pancreaticoduodenectomy for periampullary tumors.</p><p><b>METHODS</b>POSSUM and P-POSSUM scoring systems were used to retrospectively evaluate the clinical data of 432 patients with periampullar tumors who underwent pancreaticoduodenectomy in the Department of Abdominal Surgery, Cancer Hospital, Chinese Academy of Medical Sciences from January 1985 to December 2010. The predictive occurrence of postoperative complications and mortality rate were calculated according to the formula. ROC curve analysis and different group of risk factors were used to determine the discrimination ability of the two score systems, and to determine their predictive efficacy by comparing the actual and predictive complications and mortality rates, using Hosmer-Lemeshow test to determine the goodness of fit of the two scoring systems.</p><p><b>RESULTS</b>The average physiological score of the 432 patients was 16.1 ± 3.5, and the average surgical severity score was 19.6 ± 2.7. ROC curve analysis showed that the area under ROC curve for mortality predicted by POSSUM and P-POSSUM were 0.893 and 0.888, showing a non-significant difference (P > 0.05) between them. The area under ROC curve for operative complications predicted by POSSUM scoring system was 0.575. The POSSUM score system was most accurate for the prediction of complication rates of 20%-40%, showing the O/E value of 0.81. Compared with the POSSUM score system, P-POSSUM had better ability in the prediction of postoperative mortality, when the predicted value of mortality was greater than 15%, the predictive result was more accurate, and the O/E value was 1.00.</p><p><b>CONCLUSIONS</b>POSSUM and P-POSSUM scoring system have good value in predicting the mortality of patients with periampullary tumors undergoing pancreaticoduodenectomy, but a poorer value of POSSUM score system in prediction of complications. We can establish a more suitable scoring system for pancreaticoduodenectomy by modifying the score constant and weight, to better predict surgical risk and reduce the operative complications and mortality.</p>

Metastatic mucinous adenocarcinoma of the distal common bile duct, from transverse colon cancer presenting as obstructive jaundice

The patient was a 70-year-old male whose chief complaints were obstructive jaundice and weight loss. Abdominal imaging studies showed a 2.5 cm sized mass at the distal common bile duct, which was suggestive of bile duct cancer. Eccentric enhancing wall thickening in the transverse colon was also shown, suggesting concomitant colon cancer. A colonoscopy revealed a lumen-encircling ulcerofungating mass in the transverse colon, that was pathologically proven to be adenocarcinoma. The bile duct pathology was also adenocarcinoma. Pylorus-preserving pancreaticoduodenectomy and extended right hemicolectomy were performed under the diagnosis of double primary cancers. Postoperative histopathologic examination revealed moderately differentiated mucinous adenocarcinoma of transverse colon cancer, and mucinous adenocarcinoma of the distal common bile duct. Immunohistochemical staining studies showed that the bile duct cancer had metastasized from the colon cancer. The patient recovered uneventfully from surgery and will be undergoing chemotherapy for three months.