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.

Correlation of the duration of preoperative biliary drainage and postoperative complications after pancreaticoduodenectomy / 肿瘤研究与临床

Objective:To investigate the effect of the duration of preoperative biliary drainage on postoperative complications after pancreaticoduodenectomy.Methods:The clinical data of 102 patients with benign and malignant hepatopancreatic ductal periampullary tumors who underwent pancreaticoduodenectomy and preoperative biliary drainage in Beijing Friendship Hospital, Capital Medical University from January 2016 to July 2020 were retrospectively analyzed. According to the median duration of preoperative biliary drainage, the patients were divided into short-term drainage group (≤ the median duration of biliary drainage) and long-term drainage group (> the median duration of biliary drainage). The general data, the effect of biliary drainage, inflammation-related indicators and postoperative complications were compared between the two groups. Multivariate logistic regression was used to screen the risk factors related to the postoperative severe complications.Results:Of the 102 patients, 68 (66.7%) were males and 34 (33.3%) were females, with a median age of 63 years (43-80 years). The median duration of preoperative biliary drainage was 14 d. There were 68 patients in short-term drainage group and 34 patients in long-term drainage group. There were no statistically significant differences in age, gender, body mass index (BMI), hypertension, diabetes mellitus, surgery history of upper abdominal, American Society of Anesthesiologists (ASA) grade, carcinoembryonic antigen, carbohydrate antigen 125, alpha-fetoprotein, prothrombin time, pancreaticojejunostomy method, operation time, and pathological type between the two groups (all P > 0.05). However, patients in long-term drainage group had higher conversion rate, more blood loss and longer hospital stay compared with those in short-term drainage group (all P < 0.05). Before biliary drainage, alanine aminotransferase (ALT) level in short-term drainage group was higher than that in long-term drainage group ( Z = -2.59, P = 0.009), and there were no statistically significant differences in aspartate aminotransferase (AST), albumin (ALB), total bilirubin (TB) and direct bilirubin (DB) levels between the two groups before biliary drainage (all P > 0.05). After biliary drainage, DB in short-term drainage group was higher than that in long-term drainage group ( Z = -3.34, P = 0.001), and there was no statistically significant difference in ALT, AST, ALB, TB levels between the two groups (all P > 0.05). There were no statistically significant differences in the levels of white blood cells, neutrophils, lymphocytes and the ratio of neutrophils to lymphocytes between the two groups on the 1st and 3rd day after the operation (all P > 0.05). The total incidence of postoperative related complications in short-term drainage group and long-term drainage group was 63.2% (43/68), 70.6% (24/34), respectively, and the difference was statistically significant ( χ2 = 0.54, P = 0.461); the incidences of bile leakage, abdominal or gastrointestinal bleeding, intra-abdominal infection, delayed gastric emptying, all grades of pancreatic leakage, grade B and C pancreatic leakage were not statistically different between the two groups (all P > 0.05); the incidence of severe postoperative related complications in short-term drainage group was higher than that in long-term drainage group [27.9% (19/68) vs. 8.8% (3/34), χ2 = 4.90, P = 0.027]. Multivariate logistic regression analysis showed that the long-term preoperative biliary drainage was an independent protective factor for postoperative severe complications (long-term drainage vs. short-term drainage: OR = 0.253, 95% CI 0.066-0.975, P = 0.046), while BMI ( OR = 1.174, 95% CI 0.986-1.398, P = 0.071) and pathological type (benign or borderline vs. malignant tumor: OR = 0.247, 95% CI 0.043-1.419, P = 0.117) were not independent influencing factors for postoperative severe complications. Conclusions:Short-term biliary drainage (≤14 d) is a risk factor for postoperative severe complications in patients with hepatopancreatic ductal periampullary tumor undergoing preoperative biliary drainage. Preoperative biliary drainage time is not associated with postoperative total complications, pancreatic leakage, bile leakage, abdominal or gastrointestinal bleeding, intra-abdominal infection, delayed gastric emptying.

Analysis of 17 cases of Vaterian system adenomyomatous hyperplasia / 中华肝胆外科杂志

Objective:To study the features of adenomyomatous hyperplasia (AH) of the Vaterian system (common bile duct and ampulla of Vater) to help in the diagnosis and management of this disease.Methods:A retrospective analysis on the data of 17 patients who had a postoperative pathological diagnosis of AH of the Vaterian system treated from January 2005 to December 2021 at the First Medical Center of the PLA General Hospital was carried out with 12 males and 5 females, aged (58.4±11.3) years. The clinical presentations, treatment and postoperative pathology of these patients were analyzed. Patients with dysplasia of the tubular mucosal epithelium in the non-cancerous area around the AH under microscopy were included in the AH with dysplasia group ( n=8), and those without dysplasia were included in the control group ( n=9). The clinical characteristics of the two groups were compared. Results:The main clinical symptoms were abdominal pain in 8 patients, jaundice in 7 patients and fever in 2 patients. Preoperative imaging showed 10 cases of occupying lesions and 6 cases of abnormally dilated intrahepatic and extrahepatic bile ducts without obvious lesions or stones or biliary tract injury stenosis. Sixteen patients underwent radical pancreaticoduodenectomy, and 1 patient underwent extrahepatic biliary resection combined with choledochojejunostomy for bile duct obstruction due to biliary stones, 3 patients had combined malignant tumors, 1 patient had a carcinoma of AH origin at the ampulla of Vater, and the other 2 patients had neoplastic lesions in the mucosal epithelium adjacent to the AH (cholangiocarcinoma and ampullary carcinoma, respectively). There were no significant differences in age, gender, bile duct stones, cholangitis, combined carcinoma and liver function indexes between the two groups of patients with AH of the Vaterian system (all P>0.05). Conclusion:Adenomyomatous hyperplasia of the Vaterian system was difficult to distinguish preoperatively from malignant tumors basing on its clinical presentations or imaging findings. Such patients are recommended to be treated surgically.

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.

Construction and validation of the preoperative nomogram diagnosis model for pancreatic head cancer and distal cholangiocarcinoma / 中华肝胆外科杂志

Objective:To study the differences in preoperative examination indexes between pancreatic head cancer and distal bile duct cancer, and to establish a preliminary prediction model to provide reference for clinical decision-making.Methods:Retrospective analysis was conducted on 243 consecutive patients who underwent open radical pancreaticoduodenectomy from January 1, 2015 to December 31, 2019 at the Chinese PLA General Hospital. There were 177 males and 66 females, aged (58.9±8.9) years old. Based on postoperative pathological results, these 243 patients were divided into the pancreatic head cancer group ( n=113) and the distal bile duct cancer group ( n=130). The clinical data of the two groups were collected. Minimum absolute value convergence and selection operator regression were used to screen the best predictors of pancreatic head cancer. The rms package was used to construct the nomogram model, and k-fold cross was used for internal validation. Results:Seven best predictive indexes are selected: age, rate of weight loss, main pancreatic duct diameter, neutrophil/lymphocyte, DTR (DBil/TBil), carcinoembryonic antigen and CA125. Based on these indexes, a nomogram prediction model was constructed with the C-index of 0.868 after k-fold cross-validation, indicating that discrimination of the model to be acceptable. Validation using calibration curve exhibited good concordance between the predicted probability with the ideal probability ( P=0.728). Conclusion:The nomogram prediction model established in this study effectively predicted patients with pancreatic head cancer, and helped preoperatively to differentiate pancreatic head cancer from distal cholangiocarcinoma.

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.

Acute gallstone pancreatitis caused by duodenal papillary impacted stone: a study on its risk factors based on ERCP findings / 中华肝胆外科杂志

Objective:To study the risk factors of acute gallstone pancreatitis (AGP) caused by impaction of duodenal papilla stones based on ERCP findings to provide evidence on prevention of AGP caused by stone impaction.Methods:The data of 304 patients with duodenal papilla stone impaction who were treated by ERCP at the Integrated Chinese and Western Medicine Hospital of Tianjin University from January 2009 to December 2020 were analyzed retrospectively. There were 177 males and 127 females, with a median age of 65.0 years. These patients were divided into the AGP group ( n=174) and the non-AGP group ( n=130) according to whether they developed acute pancreatitis before hospitalization. The analysis was performed on perioperative data. Multivariate logistic regression analysis was used to detect risk factors of AGP in patients with duodenal papillary stone impaction. Results:Multivariate logistic analysis showed that acute cholangitis ( OR=2.114, 95% CI: 1.279-3.494, P<0.05) and impacted stones ≤5 mm ( OR=1.738, 95% CI: 1.064-2.840, P<0.05) were independent risk factors of duodenal papillary stone impaction complicated with AGP. No perforation and death related to ERCP treatment occurred in both groups. The symptom alleviating time of patients in the AGP versus the non-AGP groups was (2.67±1.19) versus (1.88±0.88) d respectively ( t=-6.321, P<0.001). Conclusion:Among patients with duodenal papilla impacted stones, acute cholangitis and impacted stones ≤5 mm were risk factors of developing AGP, and ERCP should be carried out as early as possible.

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.

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.

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)

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.

Clinical value of arterial first approach in laparoscopic pancreaticoduodenectomy for periampullary tumor / 中国医师进修杂志

Objective To investigate the clinical value of arterial first approach in laparoscopic pancreaticoduodenectomy (LPD) for periampullary tumor. Methods The data of 134 patients with periampullary tumor who had underwent LPD between February 2014 and August 2017 were collected. The patients were divided into 2 groups by random digital table method, with 67 cases in each group. The experimental group received arterial first approach LPD, and the control group received traditional approach LPD. The intraoperative, postoperative and survival situations were compared between 2 groups. Follow- up using outpatient examination and telephone interview was performed to detect the tumor- free survival up to February 2018. Results All the patients underwent successful LPD. The time of tumor resection with superior mesenteric venous invasion, volume of intraoperative blood loss and volume of blood transfusion in experimental group were significantly lower than those in control group: (215.6 ± 12.3) min vs. (263.4 ± 21.2) min, (98.9 ± 15.6) ml vs. (130.1 ± 26.7) ml and (1.4 ± 0.9) U vs. (2.9 ± 1.3) U, and there were statistical differences (P<0.05). The rate of R0 resection in experimental group was significantly higher than that in control group: 97.0％ (65/67) vs. 88.1％ (59/67), and there was statistical difference (P<0.05). There was no statistical difference in postoperative 6- month tumor- free survival rate between 2 groups (P>0.05). Conclusions Arterial first approach in LPD can significantly reduce the volumes of intraoperative blood loss and blood transfusion, shorten the time of tumor resection with superior mesenteric venous invasion, and increase the rate of R0 resection.

A Case of Concurrent Ampullary Adenoma and Gangliocytic Paraganglioma at the Minor Papilla Treated with Endoscopic Resection

A gangliocytic paraganglioma is a benign tumor of the digestive system with a very low incidence. The tumor is histopathologically characterized by a triphasic pattern consisting of epithelioid, ganglion, and spindle-shaped Schwann cells. In most cases, it occurs in the second portion of the duodenum near the ampulla of Vater. We report a case of a gangliocytic paraganglioma occurring at the minor duodenal papilla (a rare location) with a concurrent adenoma of the ampulla of Vater. Both lesions were treated simultaneously using endoscopic resection. Additionally, we have presented a literature review.

Combined application of immunohistochemical markers to identify pathologic subtypes of ampullary carcinoma and its clinical significance / 中华病理学杂志

Objective@#To investigate the expression of immunomarkers CK7, CK20, CK17, CDX2, MUC1 and MUC2 in primary adenocarcinoma of the ampulla of Vater, to explore the role of these markers in the histopathologic subclassification of ampullary carcinoma; and to provide biologic basis for precision treatment of patients with different types of ampullary carcinoma.@*Methods@#Forty-two cases of primary ampullary carcinoma were collected at Peking University People′s Hospital, from 2012 to 2018 year. There were 22 males and 20 females. Aged range 42 to 88 years old, with mean aged (62±11) years. Among the patients, 6 was high differentiation, 19 median differentiation, and 17 low differentiation. Immunohistochemical studies on the expression of CK7, CK20, CK17, CDX2, MUC1 and MUC2 were performed in 42 cases of primary ampullary carcinoma. The relationship between different ampullary carcinoma subtypes and clinicopathologic survival data was analyzed using SPSS 16.0 statistical software.@*Results@#Three histopathologic subtypes were observed. Among 42 cases, 8(19.0%)were classified as intestinal subtype, which showed a positive expression rate of 8/8 for both CK20 and CDX2, and 5/8 for MUC2. Both CK7 and CK17 were weakly expressed in one case (1/8). No expression was observed for MUC1 in this subtype. Twenty-two (52.4%,22/42) cases were classified as pancreaticobiliary subtype, which showed a positive expression rate of 100.0%(22/22) for both CK7 and MUC1, and 90.9% (20/22) for CK17. No expression was observed for CK20, CDX2 and MUC2.The remaining 12 (28.6%) cases were classified as mixed subtype, which showed variable expression patterns. The expression frequencies of these 6 immunomarkers in different subtypes of ampullary carcinoma did not correlate with various clinicopathologic factors such as patient gender and age, tumor size, histologic differentiation, pancreatic and bile duct invasion, or the depth of duodenal invasion. However, stage Ⅲ+Ⅳ diseases were more commonly seen in pancreaticobiliary type (63.6%,14/22) than intestinal type (2/8) and mixed type (3/9; χ2=6.508, P=0.039). Follow-up data showed a trend of better survival rate for patients with intestinal subtype than those with mixed and pancreaticobiliary subtypes.@*Conclusions@#Ampullary carcinoma can be subclassified into three different subtypes using a panel of six immunomarkers, especially for the identification of subtypes of poorly differentiated carcinoma. CK7, CK17 and MUC1 are major markers of pancreaticobiliary subtype, whereas CK20, CDX2 and MUC2 are useful markers for intestinal subtype. The mixed subtype variably expresses these markers. The prognosis of patients with intestinal subtype appears better than that of pancreaticobiliary and mixed subtypes. Accurate subtyping of ampullary carcinoma is clinically important to patient management and prognosis assessment.

Papillary Cannulation Facilitated by Submucosal Saline Injection into an Intradiverticular Papilla

Endoscopic retrograde cholangiopancreatography (ERCP) of the intradiverticular papilla with its invisible orifice remains challenging. Several techniques have been introduced to evert the papillary opening to facilitate cannulation. A 79-year-old woman with bile duct stones underwent ERCP, which revealed that the papilla was located inside a large diverticulum and tended to rotate inward with a trial of papillary cannulation. Submucosal papillary injection of 3 cc of normal saline was performed at 3 and 9 o’clock. Eversion and fixation of a papilla in the diverticulum with this technique allowed selective cannulation of the biliary tree. Stones were retrieved after endoscopic papillary balloon dilation without complications. She had an uneventful post-procedural course. Our findings suggest that submucosal saline injection technique is safe and effective for selective cannulation and can be recommended when cannulation is very difficult because of an intradiverticular papilla.

Endoscopic Sphincterotomy, Balloon Stone Extraction, and Basket Stone Extraction / 대한췌담도학회지

Endoscopic sphincterotomy is performed after selective cannulation to remove the gallstone. Endoscopic sphincterotomy can cause complications such as bleeding, perforation and pancreatitis. Various types of endoscopic sphincter incision method and current generators used for incisions have been developed to reduce the incidence of such complications and increase the success rate of the procedure. In addition, guidelines for the direction and extent of endoscopic sphincterotomy and incision technique are established. The method used for the removal of gallstones after the endoscopic sphincterotomy is a method using a balloon and/or a basket. This review introduces the technical methods of endoscopic sphincterotomy and discusses the clinical indications and technical methods for representative methods of effective gallstone removal.

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.