Mixed Adenoneuroendocrine Carcinoma in the Ampulla of Vater: Case Report

A mixed non-neuroendocrine neuroendocrine neoplasm is a mixed neoplasm with a neuroendocrine component combined with a non-neuroendocrine component. It has a low incidence and limited studies, but with evidence of being an aggressive entity associated with poor survival. We present the case of a 58-year-old woman admitted with clinical symptoms of abdominal pain in the left hypochondrium associated with generalized jaundice and feverish spikes with an imaging diagnosis of bile duct dilation secondary to distal choledocholithiasis. Endoscopic retrograde cholangiopancreatography (ERCP) was performed, finding a significant papilla with a neoplastic appearance, which was biopsied and histopathologically analyzed. The diagnosis of mixed carcinoma with a component of high-grade poorly differentiated neuroendocrine carcinoma and a component of mucinous carcinoma was confirmed. Therefore, we decided to schedule a pancreaticoduodenectomy.

La neoplasia neuroendocrina no neuroendocrina mixta es una neoplasia mixta con un componente neuroendocrino combinado con un componente no neuroendocrino. Esta presenta una incidencia baja y estudios limitados, pero con evidencia de ser una entidad agresiva asociada a una pobre supervivencia. Presentamos el caso de una mujer de 58 años que ingresó por un cuadro clínico de dolor abdominal en el hipocondrio izquierdo asociado a ictericia generalizada y picos febriles con diagnóstico imagenológico de dilatación de la vía biliar secundaria a coledocolitiasis distal, por lo que se realizó una colangiopancreatografía retrógrada endoscópica (CPRE) en la que se encontró una papila mayor de aspecto neoplásico a la cual se le realizó una biopsia analizada histopatológicamente y se confirmó el diagnóstico de carcinoma mixto con componente de carcinoma neuroendocrino pobremente diferenciado de alto grado y componente de carcinoma mucinoso, por lo cual se decidió programar una pancreatoduodenectomía.

Características morfológicas de la papila duodenal y su relación con complicaciones poscolangiopancreatografía retrógrada endoscópica en un hospital de Perú / Morphological Characteristics of the Duodenal Papilla and its Association with Complications Post-Endoscopic Retrograde Cholangiopancreatography (ERCP) in a Peruvian Hospital

Resumen Introducción: existen diversos factores de riesgo para presentar complicaciones poscolangiopancreatografía retrógrada endoscópica (CPRE), uno de los recientemente estudiados es la morfología de la papila duodenal. Objetivos: evaluar la asociación entre las características morfológicas de la papila duodenal y las complicaciones pos-CPRE en pacientes atendidos en el servicio de gastroenterología de un hospital de referencia de Perú. Métodos: estudio prospectivo y analítico que incluyó a 138 pacientes en los que se realizó CPRE, estableciendo relación entre el tipo de papila duodenal según la clasificación endoscópica propuesta por Haraldsson y colaboradores, y las complicaciones pos-CPRE de hasta 1 mes de seguimiento. Resultados: se incluyeron 138 pacientes, 93 mujeres (68,42 %) y 45 varones (31,58 %), con una edad promedio de 51,46 años. El tipo 1 se asoció con menor dificultad en la canulación con un odds ratio (OR): 0,42 (intervalo de confianza [IC]: 0,20-0,88). El tipo 4 presentó significativamente mayor tiempo de canulación (6,83 minutos). La tasa de pancreatitis pos-CPRE fue de 2,9 %; de sangrado, 1,45 %, y de perforación, 0,72 %. La perforación presentó asociación estadísticamente significativa con el tipo de papila (p = 0,009). El tipo 2 presentó mayores tasas de pancreatitis (9,09 %) y perforación (9,09 %) pos-CPRE. Conclusión: el tipo de papila duodenal se asocia significativamente con perforación pos-CPRE. El tipo 2 presentó tasas más altas de complicaciones.

Abstract Introduction: several risk factors exist for complications post-endoscopic retrograde cholangiopancreatography (ERCP), and the morphology of the duodenal papilla is among those recently studied. Objectives: to evaluate the association between the morphological characteristics of the duodenal papilla and post-ERCP complications in patients seen in the gastroenterology unit of a Peruvian referral hospital. Methods: a prospective and analytical study including 138 patients who underwent ERCP, establishing a relationship between the type of duodenal papilla according to the endoscopic classification proposed by Haraldsson et al. and post-ERCP complications for up to 1 month of follow-up. Results: one hundred thirty-eight patients were included, 93 were females (68.42%), and 45 were males (31.58%), with 51.46 years of mean age. Type 1 was associated with less difficulty in cannulation, with an odds ratio (OR): 0.42 (confidence interval [CI]: 0.20-0.88). Type 4 had a significantly longer cannulation time (6.83 minutes). The post-ERCP ratio for pancreatitis was 2.9%; bleeding, 1.45%, and perforation, 0.72%. The perforation showed a statistically significant association with papilla type (p = 0.009). Type 2 showed higher rates of pancreatitis (9.09%) and post-ERCP perforation (9.09%). Conclusion: the duodenal papilla type is significantly associated with post-ERCP perforation. Type 2 showed higher complication rates.

Asociación entre el tipo de papila duodenal mayor y canulación biliar difícil en centro terciario privado / Association between the type of major duodenal papilla and difficult biliary cannulation in a private tertiary center

RESUMEN Introducción : La canulación difícil en casos de colangiopancreatografía retrógrada endoscópica (CPRE) podría asociarse a diversos factores incluyendo el tipo de papila mayor, sin embargo, existen datos limitados con respecto a esta posible asociación. Objetivos : Determinar la asociación entre el tipo de papila y la canulación biliar difícil. Materiales y métodos : Se realizó un estudio retrospectivo transversal analítico en pacientes mayores de 18 años en quienes se realizó CPRE en papila virgen, de julio 2019 a abril 2021, en una institución privada. Se excluyeron las canulaciones frustras. La papila fue clasificada en base a la clasificación de Haraldsson. Se evaluó la asociación cruda y ajustada a posibles confusores entre el tipo de papila y canulación difícil. Se calcularon los riesgos relativo (RR) e intervalos de confianza al 95%. Resultados : Se incluyeron 188 pacientes. La edad media fue 55 años, el 66% de sexo femenino. La indicación más frecuente fue coledocolitiasis con 88,5%. El tipo de papila duodenal mayor más frecuente fue el tipo 1 (32%), seguido de tipo 3 (27%), tipo 2 (25%) y tipo 4 (16%). Las papilas tipo 2, 3, 4 presentaron una relación significativa con canulación difícil comparadas con la tipo 1 (p<0,001, p<0,001 y p=0,008 respectivamente). La indicación diferente a coledocolitiasis también mostró una relación significativa con canulación difícil (p<0,001). En el análisis ajustado, El RR para canulación difícil en comparación con la papila tipo 1 fue: de 2,51 (IC 95% 1,23-5,94) para la papila tipo 2, 3,72 (IC 95% 1,79-7,71) para la papila tipo 3 y 3,41 (IC 95% 1,54-7,71) para la tipo 4. La indicación distinta a la coledocolitiasis también se asoció a un mayor riesgo de canulación difícil con un RR de 2.36 (IC95% 1,57-3,56). El precorte tipo fistulotomía fue usado con mayor frecuencia en la papila tipo 3 (46%) mientras que el uso de canulótomo fue más frecuente en la papila tipo 4 (29,6%). Conclusiones : Los tipos de papila 2, 3 y 4, están asociados a mayor riesgo de canulación difícil. Ello debe ser considerado al momento de realizar la CPRE a fin de reducir el riesgo de complicaciones.

ABSTRACT Introduction : Difficult cannulation in cases of endoscopic retrograde cholangiopancreatography (ERCP) could be associated with several factors, including: type of major papilla, however, there are limited data regarding this possible association. Objectives : To determine the association between the type of papilla and difficult biliary cannulation. Materials and methods : A retrospective cross-sectional analytical study was conducted in patients over 18 years old who underwent ERCP on papilla naive, from July 2019 to April 2021, in a private institution. Unsuccessful cannulations were excluded. The papilla was classified based on Haraldsson classification. The crude association and adjusted for possible confounders between the type of papilla and difficult cannulation was evaluated. Relative risks (RR) and 95% confidence intervals were calculated. Results : 188 patients were included. The mean age was 55 years, 66% female. The most frequent indication was choledocholithiasis with 88.5%. The most frequent type of major duodenal papilla was type 1 (32%), followed by type 3 (27%), type 2 (25%) and type 4 (16%). Type 2, 3, 4 papillae showed a significant relationship with difficult cannulation compared to type 1 (p<0.001, p<0.001 and p=0.008 respectively). The indication other than choledocholithiasis also showed a significant relationship with difficult cannulation (p<0.001). In the adjusted analysis, the RR for difficult cannulation compared to type 1 papilla was: 2.51 (95% CI 1.23-5.94) for type 2 papilla, 3.72 (95% CI 1.79-7.71) for papilla type 3 and 3.41 (95% CI 1.54-7.71) for type 4. The indication other than choledocholithiasis was also associated with a higher risk of difficult cannulation with a RR of 2.36 (95% CI 1.57-3.56). The fistulotomy type precut was used more frequently in the type 3 papilla (46%), while the use of cannula was more frequent in the type 4 papilla (29.6%). Conclusions : Papilla types 2, 3 and 4 are associated with a higher risk of difficult cannulation. This should be considered when performing ERCP in order to reduce the risk of complications.

Seguimento de longo prazo de pacientes com tumor neuroendócrino da papila duodenal submetidos à papilectomia endoscópica: série de casos e revisão da literatura / Long-term follow-up of patients undergoing endoscopic papillectomy for papillary neuroendocrine tumor: a case series and literature review

ABSTRACT BACKGROUND: Duodenal papilla neuroendocrine tumors (DP-NET) are rare. Surgical therapy may be recommended for their treatment. However, they have high rates of morbidity and mortality. Endoscopic papillectomy (EP) is safe and effective for complete resection of tumors at this site. OBJECTIVE: This study aimed to describe a case series of DP-NETs resected by EP and perform a literature review. METHODS: A series of patients with DP-NETs underwent EP as primary treatment between Jan/2008 and Mar/2020 at a tertiary referral center. A comprehensive search was made on the MEDLINE primary electronic database. The search strategy was designed to find all articles related to DP-NETs published in the literature. RESULTS: Six patients underwent EP for presenting DP-NETs, four of whom were women (mean age, 63 years). The mean diameter of DP-NETs was 1.6±0.3 cm. Four of six patients were followed up, one of whom suffered relapse at the resection site after 3 months and was referred to surgery (pT3n1b) and the remaining three patients experienced no endoscopic or histological recurrence during follow-up periods of 10, 7, and 4 years, respectively. Eighteen articles were found in the literature search in MEDLINE. The articles included case reports of endoscopically treated DP-NETs. CONCLUSION: EP is safe and effective for DP-NETs that are ≤20 mm, confined to the submucosal layer, well-differentiated, and without local or remote metastasis. Adequate endoscopic follow-up and definitive surgical treatment in the presence of relapse are necessary.

RESUMO CONTEXTO: Tumores neuroendócrinos da papila duodenal (TNE-PD) são raros. A cirurgia deve ser recomendada para o tratamento. No entanto, apresentam altas taxas de morbimortalidade. A papilectomia endoscópica (PE) é segura e eficaz para a ressecção completa de tumores nesta região. OBJETIVO: Descrever uma série de casos de TNEs-PD ressecados por PE e realizar uma revisão da literatura. MÉTODOS: Pacientes com TNEs-PD submetidos a PE como tratamento primário com intenção curativa entre jan/2008 e mar/2020 em um centro de referência terciário foram estudados. Uma pesquisa abrangente foi feita no MEDLINE. A estratégia de busca foi desenhada para encontrar todos os artigos relacionados a TNEs-PD publicados na literatura, que haviam sido submetidos a PE. RESULTADOS: Seis pacientes foram submetidos a PE por apresentar TNEs-PD, sendo quatro mulheres, com média de idade de 66 anos (22-96). O diâmetro médio dos TNEs-PD foi de 1,8±0,4 cm. Quatro dos seis pacientes foram acompanhados, um dos quais sofreu recidiva no local da ressecção após 3 meses e foi encaminhado para cirurgia (pT3n1b), e os três pacientes restantes não apresentaram recorrência endoscópica ou histológica durante os períodos de acompanhamento de 10, 7 e 4 anos, respectivamente. Dezoito artigos foram encontrados no MEDLINE. Os artigos incluíram relatos de casos de TNEs-PD tratados pela PE. CONCLUSÃO: A PE é segura e eficaz para TNEs-PD ≤20 mm, confinados à camada submucosa, bem diferenciados e sem metástases locais ou remotas. Acompanhamento endoscópico adequado e tratamento cirúrgico definitivo na vigência de recidiva são necessários.

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.

Cap-assisted endoscopy increases ampulla of Vater visualization in high-risk patients.

BACKGROUND: Periampullary adenocarcinoma is a major clinical problem in high-risk patients including FAP population. A recent modification for visualizing the ampulla of Vater (AV) involves attaching a cap to the tip of the forward-viewing endoscope. Our aim was to compare the rates of complete visualization of AV using this cap-assisted endoscopy (CAE) approach to standard forward-viewing endoscopy (FVE). We also determined: (i) the rates of complications and additional sedation; (ii) the mean time required for duodenal examination; and (iii) the reproducibility among endoscopists performing this procedure. METHODS: We performed esophagogastroduodenoscopy for AV visualization in 102 > 18 years old using FVE followed by CAE. Video recordings were blinded and randomly selected for independent expert endoscopic evaluation. RESULTS: The complete visualization rate for AV was higher in CAE (97.0%) compared to FVE (51.0%) (p <  0.001). The additional doses of fentanyl, midazolam, and propofol required for CAE were 0.05, 1.9 and 36.3 mg. in 0.9, 24.5, and 77.5% patients, respectively. The mean time of duodenal examination for AV visualization was lower on CAE compared to FVE (1.41 vs. 1.95 min, p <  0.001). Scopolamine was used in 34 FVE and 24 CAE, with no association to AV complete visualization rates (p = 0.30 and p = 0.14). Three more ampullary adenomas were detected using CAE compared to FVE. Cap displacement occurred in one patient, and there was no observed adverse effect of the additional sedatives used. Kappa values for agreement between endoscopists ranged from 0.60 to 0.85. CONCLUSIONS: CAE is feasible, reproducible and safe, with a higher success rate for complete visualization compared to FVE. TRIAL REGISTRATION: ClinicalTrials.gov , NCT02867826 , 16 August 2016.

Síndrome de Lemmel: presentación de un caso / Lemmel syndrome: presentation of a Case

RESUMEN Fundamento: el duodeno corresponde a la segunda localización, con más frecuencia se presentan en los divertículos gastrointestinales después del colon. El síndrome de Lemmel también conocido como ictericia obstructiva intermitente, presencia de divertículos duodenales próximos a la papila de Váter podría favorecer al desarrollo de enfermedades pancreatobiliares. Objetivo: presentar el caso de una adulta mayor con ictericia en mucosa y piel intermitente asociada con episodios febriles no cuantificados y dolor en hipocondrio derecho. Presentación del caso: paciente de 67 años de edad, femenina, con un síndrome ictérico obstructivo, sin presencia de coledocolitiasis u otra alteración del árbol biliar distinta a la presencia de un divertículo duodenal. Por esta sintomatología es remitido a consulta de Gastroenterología del Hospital Universitario Manuel Ascunce Domenech de la provincia Camagüey. Conclusiones: el síndrome de Lemmel es una enfermedad poco frecuente que debe tenerse en cuenta como causa de ictericia obstructiva.

ABTRACT Background: the duodenum corresponds to the second location where the gastrointestinal diverticula are most frequently presented after the colon. Lemmel's syndrome, also known as intermittent obstructive jaundice, presence of duodenal diverticula near the papilla of Vater could favor the development of pancreatic biliary diseases. Objective: to present the case of an older adult with jaundice in mucosa and intermittent skin associated with febrile episodes not quantified and pain in the right hypochondrium. Case report: a 67-year-old female patient with an obstructive jaundice syndrome, without the presence of choledocholithiasis or other alteration of the biliary tree other than the presence of a duodenal diverticulum. For this symptomatology is referred to Gastroenterology consultation. Conclusions: Lemmel's syndrome is a rare pathology that must be taken into account as a cause of obstructive jaundice.

Gallbladder and extrahepatic bile duct cancers in the Americas: Incidence and mortality patterns and trends.

Trends in gallbladder cancer incidence and mortality in populations across the Americas can provide insight into shifting epidemiologic patterns and the current and potential impact of preventative and curative programs. Estimates of gallbladder and extrahepatic bile duct cancer incidence and mortality for the year 2018 were extracted from International Agency for Research on Cancer (IARC) GLOBOCAN database for 185 countries. Recorded registry-based incidence from 13 countries was extracted from IARCs Cancer Incidence in Five Continents series and corresponding national deaths from the WHO mortality database. Among females, the highest estimated incidence for gallbladder and extrahepatic bile duct cancer in the Americas were found in Bolivia (21.0 per 100,000), Chile (11.7) and Peru (6.0). In the US, the highest incidence rates were observed among Hispanics (1.8). In the Chilean population, gallbladder cancer rates declined in both females and males between 1998 and 2012. Rates dropped slightly in Canada, Costa Rica, US Whites and Hispanics in Los Angeles. Gallbladder cancer mortality rates also decreased across the studied countries, although rising trends were observed in Colombia and Canada after 2010. Countries within Southern and Central America tended to have a higher proportion of unspecified biliary tract cancers. In public health terms, the decline in gallbladder cancer incidence and mortality rates is encouraging. However, the slight increase in mortality rates during recent years in Colombia and Canada warrant further attention. Higher proportions of unspecified biliary tract cancers (with correspondingly higher mortality rates) suggest more rigorous pathology procedures may be needed after surgery.

Adenocarcinoma de papila duodenal maior - Um relato de caso / Adenocarcinoma of the major duodenal papilla - A case report

Tumores periampulares (TP) são originados a partir de estruturas próximas a Ampola de Vater e correspondem a um grupo heterogêneo de neoplasias. O adenocarcinoma da papila duodenal maior faz parte desse grupo de neoplasias. As principais manifestações são a icterícia do tipo obstrutiva, prurido, colúria, acolia fecal, além de perda ponderal e dor abdominal, associado a elevação dos níveis séricos de bilirrubina e enzimas hepáticas. O prognóstico é obscuro, sendo os carcinomas do tipo pancreaticobiliar aqueles com pior prognóstico. A ecografia endoscópica é o método mais sensível para diagnóstico e estadiamento e o tratamento de escolha é pancreaticoduodenectomia, conhecida como cirurgia de Whipple, frequentemente associada à quimioterapia adjuvante ou terapia de quimiorradiação. O presente trabalho propõe descrever um caso de adenocarcinoma da ampola de Vater em paciente de 38 anos atendida no Hospital Universitário da Universidade Federal de Juiz de Fora (HU-UFJF), abrangendo desde a sintomatologia inicial, processo diagnóstico até a conduta terapêutica. Sua relevância está em alertar os profissionais da saúde sobre a importância de elencar os tumores periampulares entre os possíveis diagnósticos diferenciais para pacientes com síndrome colestática, dado que o diagnóstico e a ressecção cirúrgica precoce são os fatores decisivos para um melhor prognóstico. (AU)

Periampular tumors (PT) originate from structures close to Vater's Ampulla and correspond to a heterogeneous group of cancers. The adenocarcinoma of the major duodenal papilla is part of this group of cancers. The main manifestations are obstructive jaundice, pruritus, choluria, fecal acholia, weight loss and abdominal pain, associated with elevated serum levels of bilirubin and liver enzymes. The prognosis is poor, with pancreaticobiliar type carcinomas having the worst prognosis. Endoscopic ultrasound is the most sensitive method for diagnosis and staging, and the best treatment is pancreaticoduodenectomy, known as Whipple surgery, often associated with adjuvant chemotherapy or chemoradiation therapy. The article proposes to describe a case of adenocarcinoma of the Vater's ampulla in a 38-year-old patient treated at the University Hospital of the Federal University of Juiz de Fora (HU-UFJF), covering from the initial symptoms, the diagnostic process and the therapeutic conduct. Its relevance is to alert health professionals about the importance of listing periampular tumors among the possible differential diagnoses for patients with cholestatic syndrome, considering that the diagnosis and early surgical resection are decisive factors for a better prognosis. (AU)

Clinicopathological features related to survival in adenocarcinoma of the Vaterian system in a Mexican population.

Adenocarcinomas of the ampulla of Vater account for 0.5% of malignant neoplasms of the gastrointestinal tract and 6% to 20% of malignant periampullary neoplasms, with most patients being candidates for elective surgery. Our objective was to evaluate the clinicopathological prognostic factors of ampullary adenocarcinomas after surgical resection in a Mexican population. From the records of the Department of Pathology at the Instituto Nacional de Cancerología, México, cases diagnosed as adenocarcinomas of the ampulla of Vater were selected over a period of 11 years, from January 2005 to September 2015. Cases with a pancreaticoduodenectomy report were included, and from each case, demographic and pathological data of the surgical specimen were obtained. Univariate and multivariate statistical analyses were performed using the log-rank test and Cox regression. Of 157 cases diagnosed as ampullary adenocarcinomas, 104 patients were excluded as not eligible for surgical treatment at the time of diagnosis. In the remaining 53 patients, a pancreaticoduodenectomy was performed. The mean age of the entire group was 55.4 years, and most were men. Intestinal-type adenocarcinomas were more frequent (77.4%) than pancreatobiliary-type (15.1%), with most being without perineural invasion, well to moderately differentiated, and less than 3 cm in size. Lymph node metastasis and age greater than 65 years had a negative impact on overall survival of the patients. The most convenient classification of malignant epithelial tumors of the Vaterian system is according to the histopathologic phenotype grouped into intestinal-, pancreatobiliary-, and mixed-type adenocarcinomas, as well as uncommon variants.

Pancreaticoduodenectomy: impact of the technique on operative outcomes and surgical mortality / Duodenopancreatectomia: impacto da técnica nos resultados operatórios e mortalidade cirúrgica

ABSTRACT Background: Pancreaticoduodenectomy (PD) is a procedure associated with significant morbidity and mortality. Initially described as gastropancreaticoduodenectomy (GPD), the possibility of preservation of the gastric antrum and pylorus was described in the 1970s. Aim: To evaluate the mortality and operative variables of PD with or without pyloric preservation and to correlate them with the adopted technique and surgical indication. Method: Retrospective cohort on data analysis of medical records of individuals who underwent PD from 2012 through 2017. Demographic, anthropometric and operative variables were analyzed and correlated with the adopted technique (GPD vs. PD) and the surgical indication. Results: Of the 87 individuals evaluated, 38 (43.7%) underwent GPD and 49 (53.3%) were submitted to PD. The frequency of GPD (62.5%) was significantly higher among patients with pancreatic neoplasia (p=0.04). The hospital stay was significantly shorter among the individuals submitted to resection due to neoplasias of less aggressive behavior (p=0.04). Surgical mortality was 10.3%, with no difference between GPD and PD. Mortality was significantly higher among individuals undergoing resection for chronic pancreatitis (p=0.001). Conclusion: There were no differences in mortality, surgical time, bleeding or hospitalization time between GPD and PD. Pancreas head neoplasm was associated with a higher indication of GPD. Resection of less aggressive neoplasms was associated with lower morbidity and mortality.

RESUMO Racional : A duodenopancreatectomia (DP) é procedimento associado com significativa morbimortalidade. Inicialmente descrita como gastroduodenopancreatectomia (GDP), a possibilidade de preservação do antro gástrico e piloro foi descrita na década de 1970. Objetivo : Avaliar a mortalidade e variáveis operatórias da DP com ou sem preservação pilórica e correlacioná-las com a técnica adotada e indicação cirúrgica. Método: Estudo de coorte histórica, baseado em análise de dados de registros médicos de indivíduos submetidos à DP entre os anos de 2012 a 2017. Foram analisadas variáveis demográficas, antropométricas e operatórias e correlacionadas com a técnica adotada (GDP vs. DP) e a indicação cirúrgica. Resultados : Dos 87 indivíduos avaliados, 38 (43,7%) foram submetidos à GDP e 49 (53,3%) à DP. A frequência de realização da GDP (62,5%) foi significativamente maior entre os pacientes com neoplasia de pâncreas (p=0,04). O tempo de internação total foi significativamente menor entre os indivíduos submetidos à ressecção por neoplasias de comportamento menos agressivo (p=0,04). A mortalidade cirúrgica foi de 10,3%, não havendo diferença entre GDP e DP. A mortalidade foi significativamente maior entre os indivíduos submetidos à ressecção por pancreatite crônica (p=0,001). Conclusão : Não houve diferenças na morbimortalidade, tempo cirúrgico, sangramento ou tempo de internação entre GDP e DP. A neoplasia de cabeça de pâncreas associou-se mais com indicação de GDP. A ressecção de neoplasias menos agressivas associou-se a menor morbimortalidade.

Retrospective cohort of pancreatic and Vater ampullary adenocarcinoma from a reference center in Mexico.

INTRODUCTION: Pancreatic ductal adenocarcinoma (PDAC) and ampulla of Vater adenocarcinomas (AVAC) are periampullary tumors. These tumors have overlapping symptoms and a common treatment, but present differences in their survival and biology. No recent studies in Mexico have been published that describe the clinicopathological characteristics of these tumors. Therefore, the aim of this study was to describe the clinicopathological characteristics of PDAC and AVAC in patients at a reference center in Mexico. METHODS: A retrospective cohort of patients with PDAC or AVAC was analyzed at our institution (July 2007 to June 2016). Inferential analysis of the clinical data was performed with Student's t-test or a χ2 test with odds ratios (OR) and confidence intervals (CI), depending on the variables. Overall survival was compared using Kaplan-Meier curves with log-rank p values. RESULTS: Forty patients with PDAC and 76 with AVAC were analyzed, including 77 females and 39 males with a mean age of 60.6 years and a mean evolution time of 5.7 months. PDAC patients had more abdominal pain, a larger tumor size and more advanced stages than AVAC patients. In contrast, AVAC patients had more jaundice, a higher percentage of complete resections and higher overall survival. Up to 70% of patients were overweight. PDAC cohort included a higher proportion of smokers. CONCLUSIONS: Our cohort was slightly younger, had a larger percentage of females, and a greater percentage of obese patients than those in many international reports. A high proportion of PDAC patients are diagnosed in advanced stages and have a low likelihood of resectability.

Immunohistochemical Predictors for Intestinal and Pancreatobiliary Types of Adenocarcinoma of The Ampulla of Vater.

OBJECTIVES: To investigate immunohistochemical predictors for intestinal and pancreatobiliary types of adenocarcinoma of ampulla of Vater and identify clinicopathological characteristics associated with the histological types and patient survival. METHODS: Immunohistochemical markers included MUC1, MUC2, MUC5AC, CDX2, CK7, and CK20. The data were analyzed by univariate and multivariate methods. The two-step cluster method was used to determine the best immunohistochemical markers to discriminate the intestinal from the pancreatobiliary type. RESULTS: This study identified 9 (33.3%) intestinal and 21 (66.7%) pancreatobiliary tumors. CK7 and CDX2 achieved the highest value (= 1) as predictor markers, while CK20, MUC1, and MUC2 showed degrees of importance equal to 0.77, 0.71, and 0.68, respectively. MUC5AC did not reach 0.50 of importance. In the univariate analysis, lymph node involvement, staging (TNM), and angiolymphatic and perineural invasions were associated with histological types. The independent clinicopathological variable in the multivariate model to predict the histological type was angiolymphatic invasion (p = 0.005), OR = 17 (95% CI 2.33 to 123.83). The final model showed positive nodes (N1) associated with shorter survival (HR = 9.5; p = 0.006). Overall survival at 12, 36, and 60 months was 88.5, 67.0, and 47.6%, respectively. CONCLUSIONS: CDX2 and CK7 were the immunohistochemical markers that best discriminated the intestinal from the pancreatobiliary type. Lymph node involvement had a high impact on survival and proved to be more frequent in the pancreatobiliary type.

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.

O uso da pinça de biópsia como técnica auxiliar na visualização da papila duodenal maior utilizando-se o esofagogastroduodenoscópio de visão frontal / The use of the forceps biopsy as an auxiliary technique for the visualization of the major duodenal papilla using the foward-viewing upper endoscopy

ABSTRACT BACKGROUND: - Conventional esophagogastroduodenoscopy is the best method for evaluation of the upper gastrointestinal tract, but it has limitations for the identification of the major duodenal papilla, even after the use of the straightening maneuver. Side-viewing duodenoscope is recommended for optimal examination of major duodenal papilla in patients at high risk for lesions in this region. OBJECTIVE: To evaluate the use of the biopsy forceps during conventional esophagogastroduodenoscopy as an additional tool to the straightening maneuver, in the evaluation of the major duodenal papilla. METHODS: A total of 671 patients were studied between 2013 and 2015, with active major duodenal papilla search in three endoscope steps: not straightened, straightened and use of the biopsy forceps after straightening. In all of them it was recorded whether: major duodenal papilla was fully visualized (position A), partially visualized (position B) or not visualized (position C). If major duodenal papilla was not fully visualized, patients continued to the next step. RESULTS: A total of 341 were female (50.8%) with mean age of 49 years. Of the 671 patients, 324 (48.3%) major duodenal papilla was identified in position A, 112 (16.7%) in position B and 235 (35%) in position C. In the 347 patients who underwent the straightening maneuver, position A was found in 186 (53.6%), position B in 51 (14.7%) and position C in 110 (31.7%). Of the 161 remaining patients and after biopsy forceps use, position A was seen in 94 (58.4%), position B in 14 (8.7%) and position C in 53 (32.9%). The overall rate of complete visualization of major duodenal papilla was 90%. CONCLUSION: The use of the biopsy forceps significantly increased the total major duodenal papilla visualization rate by 14%, reaching 604/671 (90%) of the patients (P<0.01) and it can be easily incorporated into the routine endoscopic examination of the upper gastrointestinal tract.

RESUMO CONTEXTO: Esofagogastroduodenoscopia convencional é o melhor método para avaliação do trato gastrointestinal superior, mas apresenta limitações para identificação da papila duodenal maior, mesmo após emprego da manobra de retificação. Exame completo da papila duodenal maior está indicado para pacientes de alto risco para adenocarcinoma da papila duodenal maior. OBJETIVO: Avaliar a utilização da pinça de biópsia durante esofagogastroduodenoscopia convencional como ferramenta adicional à manobra de retificação na avaliação da papila duodenal maior. MÉTODOS: Foram estudados 671 pacientes entre 2013-2015 com busca ativa da papila duodenal maior em três etapas: endoscópio não retificado, endoscópio retificado e uso da pinça de biópsia após retificação. Em todas se registrou: se a papila duodenal maior foi totalmente visualizada (posição A), se parcialmente visualizada (posição B) ou se não visualizada (posição C). Caso a papila duodenal maior não tenha sido completamente visualizada, o paciente foi direcionado para a etapa seguinte. RESULTADOS: Um total de 341 era do sexo feminino (50,8%) com idade média de 49 anos. Dos 671 pacientes, em 324 (48,3%) a papila duodenal maior foi identificada na posição A, 112 (16,7%) em posição B e, 235 (35%) em posição C. Dos 347 pacientes submetidos à manobra de retificação, posição A foi encontrada em 186 (53,6%), posição B em 51 (14,7%) e posição C em 110 (31,7%). Dos 161 pacientes restantes que utilizaram a pinça de biópsia, posição A foi vista em 94 (58,4%), posição B em 14 (8,7%) e posição C em 53 (32,9%). A taxa acumulativa de visualização completa da papila duodenal maior foi de 90%. CONCLUSÃO: O uso da pinça de biópsia aumentou a taxa de visualização completa da papila duodenal maior em 14%, alcançando 604/671 (90%) dos pacientes (P<0,01) avaliados e pode ser facilmente incorporada aos exames endoscópicos de rotina do trato gastrointestinal superior.

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.

Factores asociados con la supervivencia al año de pacientes operados con cáncer ampular o de páncreas / Factors associated with survival a one year of operated patients with cancer ampullary or pancreas

RESUMEN Objetivo Establecer los factores asociados a la supervivencia a un año de los pacientes operados con tumores de páncreas y ampulares. Materiales y Métodos Estudio descriptivo retrospectivo de una cohorte de pacientes con aplicación de análisis de supervivencia, basado en información secundaria. Se estimaron frecuencias absolutas y relativas para las variables cualitativas; y medidas de tendencia central y dispersión para las variables cuantitativas. La supervivencia global se estableció por medio del método Kaplan-Meier, en el análisis bivariado se utilizó la prueba Log-Rank y para establecer los factores asociados a la supervivencia, se efectuó un modelo de riesgos proporcionales de Cox. Resultados La supervivencia a un año de los 70 pacientes operados fue del 62,5 %. Los factores asociados a la supervivencia ajustada fueron la sepsis, sangrado posoperatorio, antígeno carcinoembrionario, trasfusión de glóbulos rojos, sangrado intraoperatorio, cirugías previas de abdomen y edad. Conclusiones El conocimiento que se obtuvo de la supervivencia a un año de los pacientes operados con cáncer de páncreas y ampular, permitirá crear estrategias encaminadas a la intervención de los factores asociados, y generar información que pueda conllevar a mejorar el pronóstico de los pacientes.(AU)

ABSTRACT Objective Determine the factors associated with survival a one year of patients operated with ampullary and pancreatic tumors. Materials and Methods A retrospective descriptive study of a cohort of patients with application of survival analysis based on secondary data. Absolute and relative frequencies for qualitative variables were estimated; and measures of central tendency and dispersion for quantitative variables. Overall survival was estimate by the Kaplan-Meier method, in the bivariate analysis was used the Log-Rank test and to establish the factors associated with survival was made a model Proportional Hazards of Cox. Results The survival a one year of 70 operated patients was 62,5 %. Factors associated with survival were adjusted sepsis, postoperative bleeding, carcinoembryonic antigen, red cell transfusion, intraoperative bleeding, previous abdominal surgeries and age. Conclusions The knowledge obtained from the survival a one year of patients operated with pancreatic and ampullary cancer, will create intervention strategies associated factors, generate more information that may lead to improve prognosis and reduce mortality.(AU)

Manejo actual del adenoma de la ampolla de Vater / Current management of adenomas of the ampulla of Vater

A lo largo de la historia, el manejo de los tumores periampulares, malignos o benignos, ha sido la cirugía radical. Sin embargo, la gran complejidad de estos procedimientos, sus complicaciones y su alto índice de mortalidad hicieron impulsar el desarrollo de nuevas técnicas quirúrgicas mínimamente invasivas. Se presenta el caso clínico de una paciente de 55 años, con hallazgo incidental de un adenoma de la ampolla de Vater en una endoscopia de vías digestivas altas, T1N0M0, manejado con papilectomía endoscópica, sin complicaciones y con márgenes negativos en el estudio histopatológico de la pieza quirúrgica. La papilectomía endoscópica, fue reportada por primera vez por Ponchon, et al., en 1989. La presentación clínica del adenoma de la ampolla de Vater es asintomática y su diagnóstico es incidental en la endoscopia de vías digestivas altas. El diagnóstico de este tipo de neoplasia se hace teniendo en cuenta la apariencia endoscópica y la histología; por lo tanto, es vital la utilización de un duodenoscopio lateral para poder tener una evaluación completa de la papila. Es importante practicar una ultrasonografía endoscópica biliopancreática, ya que esta determina la profundidad de la lesión y, además, permite descartar adenomegalias locales o regionales. La papilectomía endoscópica es un procedimiento que requiere de manos experimentadas para disminuir el riesgo de complicaciones.

Introduction: Historically, the management of periampullary tumors, malignant or benign, has been radical surgery, starting with Alessandro Codivilla, who in 1898 described the surgical technique for the performance of pancreaticoduodenectomy. Later, in 1899, William Halsted performed the first transduodenal ampullectomy. The complexity of these procedures, the associated complications and high mortality stimulated the development of novel minimally invasive surgical techniques. Case report: Fifty five year old female with incidental finding of an adenoma of the ampulla of Vater at endoscopy of the upper digestive tract, T1N0M0, managed by endoscopic papillectomy, without complications; surgical pathology reported negative margins. Discussion: Endoscopic papillectomy was first reported by Ponchon et al in 1989. The presence of an adenoma of the ampulla of Vater remains asymptomatic and the diagnosis is an incidental finding during upper digestive tract endoscopy. The diagnosis of this neoplasm is made by the endoscopic appearance and histopathology. It is imperative to use a lateral duodenoscope in order to have full assessment of the papilla. It is also important performing pancreatic biliary endosonography, as this determines the depth of the lesion and also rules out local or regional lymphadenopathy. Endoscopic papillectomy is a procedure that requires experienced hands, to avoid complications such as perforation, cholangitis, papillary stenosis, bleeding and pancreatitis, the last two being the most common with an incidence up to 25-30%. Conclusions: Randomized clinical trials are required to substantiate the benefit of endoscopic papillectomy in malignant pathology, and also there is need to develop management guidelines.

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.

Quistes del colédoco / Choledochal cysts

Los quistes del colédoco son una condición médica poco frecuente, la cual se caracteriza por presentar dilatación de la vía biliar intrahepática y extrahepática; se postulan en su etiología la anomalía de la unión pancreático-biliar, lo que favorece el reflujo del jugo pancreático al árbol biliar, y la aganglionosis del árbol biliar. Tiene una amplia gama de presentación de síntomas, y entre los principales se encuentran el dolor abdominal, la ictericia y una masa abdominal palpable. Son lesiones premalignas y, por ende, el tratamiento de elección es la resección quirúrgica completa con seguimiento a largo plazo.

Choledochal cysts are rare pathological conditions characterized by dilatation of the intrahepatic and extrahepatic bile ducts. Postulated etiologies are an abnormal pancreato-biliary junction favoring reflux of pancreatic juice to the biliary tree, and aganglionosis of the biliary tree. They have a wide range of presenting symptoms, the main ones being abdominal pain, jaundice and evident abdominal mass. Choledochal cysts are premalignant lesions, and therefore the treatment of choice is complete surgical resection, which demands long-term follow-up.