Enteroblastic adenocarcinoma of the ampulla of Vater / Adenocarcinoma enteroblástico de la ampolla de Vater

Adenocarcinoma with enteroblastic differentiation is a rare histologic subtype of adenocarcinoma of the gastrointestinal tract that shows unique histologic and immunohistochemical features that resemble fetal intestinal epithelium. This histological subtype has been widely described in the stomach, where it most frequently appears, but, in other locations, it is misdiagnosed because of the poor experience in routine diagnostic setting. Here we present a case of an 87-year-old male with an adenocarcinoma of the ampulla of Vater with enteroblastic differentiation with a literature review of the cases described of this subtype in this location to date. The anatomical peculiarity of the ampulla, joined with the infrequent nature of this histological subtype, makes this case of great interest to aid to better characterize the biological behavior of these tumors. (AU)

El adenocarcinoma con diferenciación enteroblástica es un subtipo histológico poco frecuente de adenocarcinoma gastrointestinal que muestra características histológicas e inmunohistoquímicas únicas que se asemejan al epitelio intestinal fetal. Este subtipo histológico ha sido ampliamente descrito en el estómago, donde aparece con mayor frecuencia, pero en otras localizaciones es mal diagnosticado debido a la poca experiencia en el diagnóstico de rutina. Presentamos un caso de un varón de 87 años con adenocarcinoma de ampolla de Vater con diferenciación enteroblástica, junto a una revisión bibliográfica de los casos descritos de este subtipo en esta localización hasta el momento. La peculiaridad anatómica de la ampolla, sumada al carácter poco frecuente de este subtipo histológico, dotan a este caso de gran interés para ayudar a caracterizar mejor el comportamiento biológico de estos tumores. (AU)

Opciones en el tratamiento quirúrgico de la neoplasia neuroendocrina de la ampolla de Váter: experiencia en un centro de referencia / Surgical options for the treatment of neuroendocrine neoplasms of the ampulla of Vater: a reference centre experience

Introducción: El objetivo del estudio fue analizar los resultados del tratamiento quirúrgico de las neoplasias neuroendocrinas (NNE) ampulares mediante Ampulectomía transduodenal (ATD) y duodenopancreatectomía cefálica (DPC), en un centro de referencia en patología hepatobiliopancreática. Método: Estudio retrospectivo, observacional, incluyendo los pacientes intervenidos de NNE de páncreas y/o duodenales en una unidad de referencia en patología hepatobilipancreática y registrados prospectivamente entre el 1 de enero de 1993 y el 30 de septiembre de 2021. Para aquellos parámetros no presentes, se realizó una búsqueda retrospectiva. Se analizaron datos demográficos, clínicos, analíticos y anatomopatológicos. Se realizó un análisis descriptivo. La supervivencia global y libre de enfermedad se calculó mediante curvas de Kaplan-Meier y el test de log-rank. Resultados: De 181 pacientes intervenidos de NNE de páncreas y/o duodenales, solo nueve se localizaban en la ampolla de Váter, lo que representa 4,9% de todos los NNE pancreáticos y/o duodenales. Se realizó DPC en seis pacientes, mientras que a tres se les practicó ATD. Se observó mayor tiempo quirúrgico y más complicaciones en el grupo DPC. No hubo diferencias en la estancia hospitalaria. La supervivencia global y libre de enfermedad a cinco años del grupo DPC respecto a la ATD fue de 83,3 vs. 100% y del 50 vs. 100%, respectivamente. Conclusiones: Las NNE ampulares sin afectación locorregional ni factores de riesgo, pueden ser tratadas mediante cirugías preservadoras como la ATD. (AU)

Introduction: The main objective of this study was to analyse the results of the surgical treatment of ampullary neuroendocrine tumours (NET) based on transduodenal ampullectomy and pancreatoduodenectomy, in a reference centre in hepatobiliopancreatic pathology. Method: Retrospective, observational study, including all patients operated on for pancreatic and/or duodenal NET in a reference unit of hepatobiliopancreatic pathology and prospectively registered between January 1st, 1993 and September 30th,2021. For those parameters not present, retrospective research was performed. Demographic, clinical, analytical and pathological data were analysed. A descriptive study was carried out. Overall and disease-free survival was calculated using Kaplan-Meier curves and the Log-Rank test. Results: Of 181 patients operated on for pancreatic and/or duodenal NET, only 9 were located in the ampulla of Vater, which represents 4.9% of all pancreatic and/or duodenal NET. Pancreatoduodenectomy (PD) was performed in 6 patients, while 3 patients underwent transduodenal ampullectomy (TDA). Longer surgical time and more postoperative complications were observed in the PD group. There were no differences in hospital stay. Overall and disease-free survival at 5 years in the PD group compared to ATD was 83.3% vs. 100% and 50% vs. 100%, respectively. Conclusions: Ampullary NET without locorregional involvement or risk factors, can be treated by conservative surgeries such as transduodenal ampullectomy. (AU)

Eficacia del endoscopio frontal asistido por cap como método para la evaluación de la ampolla de Vater / Efficacy of cap-assisted forward-viewing endoscopy as a method for the evaluation of the ampulla of Vater

Introducción: la evaluación endoscópica de la ampolla de Vater (AV), aunque recomendada de forma rutinaria, no siempre es posible debido a su configuración anatómica, que puede ocultarla del campo visual del endoscopio frontal. Se ha propuesto como una alternativa eficaz el usoañadido de un cap distal al endoscopio frontal para facilitar la examinación de esta estructura.Objetivos: determinar la eficacia del uso del endoscopio frontal asistido por cap para la valoración completa del AV. Se valoran la morfología del AV, los tiempos de búsqueda y total del procedimiento, así como la seguridad de la técnica.Métodos: estudio prospectivo de un solo brazo con inclusión de los pacientes en quienes se realizó una endoscopia alta electiva. Se excluyeron los pacientes con neoplasia avanzada, anatomía modificada, diagnóstico de estenosis u obstrucción del tracto digestivo superior.Resultados: se analizaron 90 pacientes, 36 hombres (40 %) y 54 mujeres (60 %), un 15,5 % del total con antecedente de un síndrome hereditario para cáncer de colon. El éxito técnico del endoscopio frontal + cap fue del 98,8 %. El AV se clasificó como tipo 1 (clásica) en el 49,4 %; tipo 2 (plana), en el 16,8 %; tipo 3 (protuberante), en el 11,2 %; y tipo 4 (anillada), en el 22,4 %. El tiempo promedio de búsqueda fue de 37,7 segundos (s) (DE ± 31,6), con un tiempo total procedimiento de 487,4 s (DE ± 206,2). No se reportaron eventos adversos.Conclusiones: el uso combinado del endoscopio frontal y cap distal es una técnica efectiva y segura para la visualización completa y caracterización morfológica del AV. (AU)

Ampuloma en paciente con antecedente de bypass gastroyeyunal en Y de Roux / Ampulloma in a Patient with a History of Roux-en-Y Gastrojejunal Bypass

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Hemosuccus pancreaticus with a variant of separate orifices in the duodenal papilla treated with a covered self-expandable metal stent

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Laparoscopic versus open pancreatoduodenectomy: a meta-analysis of randomized controlled trials

INTRODUCTION: the evidence with regard to the benefit of laparoscopic surgery for pancreatoduodenectomy is conflicting. The aim of this meta-analysis was to compare the short-term outcomes in patients undergoing laparoscopic or open pancreatoduodenectomy via randomized controlled trial studies. METHODS: PubMed, Embase and Cochrane Library databases were searched for studies addressing laparoscopic versus open pancreatoduodenectomy up to February 2019. Only randomized controlled trial studies were included. RESULTS: three randomized controlled trial studies were identified, which included a total of 224 patients. Statistically significant differences were found with regard to estimated blood loss in favor of laparoscopic pancreatoduodenectomy (WMD, -150.9 ml; 95% CI, -167.61 to -134.18; p < 0.001) but with longer operative time (WMD, 97.66 min; 95% CI, 21.28 to 174.05; p = 0.01). No significant differences were found for severe postoperative complications (defined as Clavien-Dindo grade ≥ III complications), complication-related mortality within 90 days, blood transfusion requirements, length of hospital stay, postoperative pancreatic fistula, postpancreatectomy hemorrhage, bile leakage, delayed gastric emptying, surgical site infection, readmission rate, reoperation rate, harvested lymph nodes and R0 resection rate. CONCLUSIONS: the perioperative safety of laparoscopic pancreatoduodenectomy, which may have an advantage of lower estimated blood loss, is comparable to that of open pancreatoduodenectomy. Currently, a small volume of cases may be an important reason that affects the evaluation between laparoscopic and open pancreatoduodenectomy. Further evaluation of laparoscopic pancreatoduodenectomy will require large randomized control trials

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Endoscopic papillary large balloon dilation with minor sphincterotomy for periampullary diverticular papilla

Background To observe the outcome of endoscopic papillary large balloon dilation (EPLBD) with minor sphincterotomy (mEST) for periampullary diverticular papilla related to stone removal. Methods Patients with confirmed periampullary diverticulum (PAD) during stone removal from May 2016 to April 2018 were reviewed retrospectively. The Chi-square test with Yates correction or Fisher's exact test was used for the analysis of categorical data and a normality test was applied for continuous data. Results A total of 154 consecutive patients (89 males and 65 females, aged 51-87 years) with confirmed PAD during stone removal were included in the study. Cases were divided into the conventional EST group (n = 79) and the mEST plus EPLBD group (n = 75). The number of patients with an initial treatment success was greater in the EPLBD+mEST group compared with the EST group (96% vs 86.1%, p=0.03) and the procedure time for EPLBD+mEST was shorter than that for EST alone (46.1+/-13.7 min vs 53.3+/-11.6 min, p=0.01). The rate of complications in the EPLBD+mEST group was lower than in the EST group (17.3% vs 32.9%, p=0.04). When PAD was >15 mm, the initial success rate was higher (92.6% vs 73.9%, p=0.04) and the rate of overall complications was lower (14.8% vs 41.7%, p=0.03) in the EPLBD+mEST group than those in the EST group. Although, this was similar when PAD was <15 mm. Conclusion EPLBD+mEST might be safer and more effective than conventional EST alone for stone removal in the presence of PAD

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Diagnostic yield of endoscopic ultrasonography for dilation of common bile duct of indeterminate cause

Introduction: with the widespread use of abdominal imaging, common bile duct (CBD) dilation is a common problem in the daily practice. However, the significance of a dilated CBD as a predictor of underlying disease has not been well elucidated and there are currently no guidelines for its approach. Methods: this was a retrospective study of patients who underwent endoscopic ultrasonography (EUS) from 2010 to 2017 due to a dilated CBD detected by transabdominal ultrasonography TUS (CBD ≥ 7 mm) or computed tomography (CT) (CBD ≥ 10 mm), with no identified cause (n = 56). The aims were to assess the diagnostic yield of EUS and to identify predictors for a positive EUS. Results: the majority of patients (n = 39) had normal findings on EUS. Abnormal EUS findings were found in 30% (n = 17) of the patients, which included choledocholithiasis (n = 6), ampuloma (n = 3), choledochal cyst (n = 2), benign CBD stenosis (n = 2), cyst of the head of the pancreas (n = 1), cholangiocarcinoma (n = 1), chronic pancreatitis (n = 1) and CBD compression due to adenomegaly (n = 1). Factors that positively related with findings on EUS were increased levels of gamma glutamyl transferase (331 U/l vs 104 U/l, p = 0.039), alkaline phosphatase (226 U/l vs 114 U/l, p = 0.041), total bilirubin (TB) (6.5 g/dl vs 1.2 g/dl, p = 0.035) and the presence of signs/symptoms (p = 0.042). Of the 21 patients (38%) who were asymptomatic with normal liver biochemical tests, four (19%) had findings on EUS. Conclusions: the majority of patients with a dilation of the CDB have a normal EUS. Increased cholestasis enzymes, increased TB and the presence of signs and symptoms are predictors of a positive EUS

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Endoscopic biliary drainage in unresectable biliary obstruction: the role of endoscopic ultrasound-guidance in a cohort study

Background and study aims: the use of endoscopic ultrasound-guided biliary drainage (EUS-BD) has increased in cases of failed endoscopic retrograde cholangiopancreatography (ERCP) and there are some concerns. The main aim of the study was to determine the role of EUS-BD in a palliative case cohort. The secondary aim was to compare the efficacy, safety and survival of EUS-BD and ERCP procedures. Patients and methods: this was an observational study at a single tertiary institution, with a consecutive inclusion from January 2015 to December 2016. The inclusion criteria were unresectable tumors of the biliopancreatic region with an indication of BD. Statistical comparison analysis was performed between the ERCP and EUS-BD groups. The incidence between groups was compared using the Chi-square and Fisher exact tests. The log rank test was used to compare the risk of death. Results: fifty-two cases with an indication of palliative BD were included in the study. Transpapillary drainage via ERCP was possible in 44 procedures and EUS-BD was required in eight cases; 15.4% of the cohort and seven using lumen apposing metal stent (LAMS). The technical and clinical success of global endoscopic BD was 100% and 88.5% (ERCP: 84.6% and 78.9%; EUS-BD: 100% and 62.5%, respectively). Pancreatitis was the most frequent adverse event (AE) in the ERCP group (9.62%) and bleeding in the EUS-BD (25%). There were fatal AEs in ERCP (1.9%) and EUS-BD (25%) cases. Patient survival was higher with ERCP transpapillary stents compared to EUS-guided stents, which was statistically significant (p = 0.007). Conclusions: the requirement of EUS-BD in palliative biliopancreatic pathology is not marginal. EUS-BD is associated with a lower survival rate and a higher rate of fatal AE, which argues against its use as a first choice procedure

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Carcinosarcoma of the ampulla of Vater

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Pancreatobiliary maljunction: association with gallbladder cancer

Background: Pancreatobiliary maljunction is a rare disease characterized by the junction of the pancreatic and biliary ducts outside of the duodenal wall, which normally results in a large common duct. As a result, there is a greater risk of acute pancreatitis and cancer of the gallbladder and biliary tract. Case report: We present the case of a 43-year-old female diagnosed with a pancreatobiliary maljunction and an associated stenosis of the bile duct, secondary to an episode of acute pancreatitis. She underwent several endoscopic retrograde cholangiopancreatography procedures over the course of three years, without improvement of the stenosis, and therefore a surgical approach was taken. Prior to the surgical intervention, magnetic resonance imaging showed the presence of an 11-mm polyp in the gallbladder. A histological study of the surgical sample identified intramucosal adenocarcinoma over a tubular adenoma of the gallbladder. Discussion: Pancreatobiliary maljunction can be considered as a premalignant entity due to the risk of developing cancer of the biliary tree and gallbladder. Therefore, these patients should undergo a prophylactic intervention, despite being asymptomatic

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Ampulectomía transduodenal laparoscópica por tumor ampular / Laparoscopic transduodenal ampullectomy for ampullary tumor

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Factores pronósticos en el adenocarcinoma de ampolla duodenal / Prognostic factors in adenocarcinoma of the ampulla of Vater

Introducción: El adenocarcinoma de ampolla duodenal (ampolla de Vater) parece un tumor de comportamiento menos agresivo que otros de la región pancreatobiliar. Nuestro estudio busca definir factores que condicionan el pronóstico de este tipo de neoplasia. Material y métodos: Revisión retrospectiva de los pacientes operados por adenocarcinoma de ampolla duodenal en un solo centro. Se ha valorado el intervalo libre de progresión y la supervivencia global como variables de resultado. Resultados: Un total de 24 pacientes fueron sometidos a una duodenopancreatectomía cefálica por un adenocarcinoma de ampolla duodenal. Un 54,2% eran mujeres y la edad media fue 72,5 (45-85). El principal tipo histológico fue el intestinal (50%), seguido del biliopancreático (37,5%). El resto fueron mucinosos. Solo el 8,3% de los casos fueron poco diferenciados. Se detectó invasión vascular e infiltración perineural en el 31,8 y 20,8% de los casos, respectivamente. Un 54,1% de los casos no tenían afectación ganglionar al diagnóstico y la mayoría de los tumores eran T1 o T3 (39,1 y 43,5%, respectivamente). Un 34,8% de los pacientes recayeron, sobre todo a nivel ganglionar local (62,5% de las recaídas) y todos ellos fallecieron por el tumor, en su mayoría en el primer año tras el diagnóstico. El estudio multivariable mediante regresión de Cox demostró que el único factor que condicionaba un menor intervalo libre de progresión y supervivencia global de forma independiente era el estadio N. Conclusiones: La afectación ganglionar es el factor más importante como predictor de pronóstico en esta neoplasia

Introduction: Ampullary adenocarcinoma seems less aggressive than other pancreato-biliary neoplasms. The aim of this study is to define determining prognostic factors. Material and methods: Retrospective case series from a large tertiary Hospital including all patients diagnosed with ampullary adenocarcinoma who underwent cephalic pancreatoduodenectomy with curative intent. Outcome variables have been progression free survival and overall survival. Results: 24 patients were included. 54.2% were females and the mean age was 72.5 (45-85). Most cases were of intestinal type (50%), followed by pancreatobiliary (37.5%) and mucinous. Only 8.3% were high histopathological grade. Vessel invasion was detected in 31.8% of the cases and perineural infiltration in 20.8%. A large percentage of cases showed no lymph node involvement at the time of diagnosis (54.1%). Most cases were stage T1 or T3 (39.1 y 43.5%, respectively). 34.8% of the patients recurred, mainly in regional lymph nodes (62.5% of the recurrences) and they all died of tumor, mainly during the first year after diagnosis. Multivariate analysis with Cox regression model revealed that only lymph node involvement was independently associated to a shorter disease free progression interval and overall survival. Conclusions: Lymph node involvement was the most important predictive factor for ampullary adenocarcinoma in this series

Pancreaticoduodenectomy for periampullary tumours: a review article based on Surveillance, End Results and Epidemiology (SEER) database

Introduction: This study set to examine relative survival of patients with periampullary cancers undergoing pancreaticoduodenectomy (PD). Methods: Using the Surveillance, End Results and Epidemiology (SEER) database, this study identified 9877 patients with non-metastatic pancreatic adenocarcinoma who underwent PD between 2004 and 2013. Results: Ampullary carcinomas have the best survival among periampullary malignancies. Lymph node ratio is a significant prognostic factor, even when stratified by tumour types. Patients receiving adjuvant radiotherapy following PD have superior survival than patients without radiotherapy (median 25 vs 20 months, p < 0.001), particularly ductal adenocarcinoma (HR: 0.74, CI95% 0.69-0.78; p < 0.001), cholangiocarcinoma (HR: 0.75, CI95% 0.59-0.97; p = 0.027), and ampullary carcinoma (HR: 0.79, CI95% 0.64-0.98; p = 0.029) with greatest survival benefit at 1-year postresection. Conclusion: Future studies aiming to further define genetic signatures of individual periampullary cancers would allow a personalised therapeutic approach in improving survival

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Adjuvant radiotherapy following pancreaticoduodenectomy for ampullary adenocarcinoma improves survival in node-positive patients: a propensity score analysis

Introduction: This study aimed to evaluate the impact of adjuvant radiotherapy in patients undergoing pancreaticoduodenectomy (PD) for ampullary adenocarcinoma. Methods: Using the Surveillance, Epidemiology, and End Results, patients with non-metastatic ampullary adenocarcinoma between 2004 and 2013 were identified. Cancer-specific survival and overall survival were estimated using Kaplan-Meier and Cox regression to obtain adjusted hazard ratio of survival. Results: In this study, 1106 patients with ampullary adenocarcinoma were identified, of which 27% received adjuvant radiotherapy and the remaining 73% (803/1106) patients did not receive any adjuvant radiotherapy. In the matched cohort, there were still no significant difference in CSS (median 41 vs 35, p = 0.28) and OS (median 32 vs 30, p = 0.26) between patients receiving adjuvant radiotherapy and those under observation alone. However, in patients with N2 (Fig. 4) disease, both CSS (median 27 vs 19 months, p = 0.0044) and OS (median 23 vs 17 months, p = 0.0091) were significantly longer for patients receiving adjuvant radiotherapy. Conclusion: In summary, adjuvant radiotherapy following PD for ampullary adenocarcinoma significantly improves survival in patients with N2 disease. Future studies defining "high-risk" groups using larger cohorts will enable reliable appraisal on the benefit of adjuvant radiotherapy to allow for a more personalized approach in treating patients

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Tender red subcutaneous nodules in an adult female: a challenging diagnosis

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