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Objectives: To predict tumor recurrence in patients who underwent surgical resection of ampullary adenocarcinoma using preoperative magnetic resonance (MR) imaging findings combined with clinical findings. Methods: In this multicenter study, a total of 113 patients (mean age, 62.9 ± 9.8 years; 58 men and 55 women) with ampullary adenocarcinoma who underwent preoperative MR imaging and surgery with margin-negative resection between 2006 and 2017 were retrospectively included. The MR imaging findings were evaluated by two radiologists. Preoperative clinical findings were obtained. Cox proportional regression analyses were used to identify the independent prognostic factors for recurrence-free survival (RFS). A nomogram was created based on the multivariable analysis and was internally validated. Results: Multivariable analysis revealed that presence of infiltrative tumor margin (hazard ratio [HR]: 2.18, p = 0.019), adjacent organ invasion (HR: 3.31, p = 0.006), adjacent vessel invasion (HR: 5.42, p = 0.041), peripancreatic lymph node enlargement (HR: 2.1, p = 0.019), and jaundice (HR: 1.93, p = 0.043) were significantly associated with worse RFS of ampullary adenocarcinoma after surgical resection. These MR imaging and clinical findings were used to construct a nomogram. On internal validation, the calibration plots showed excellent agreement between the predicted probabilities and the actual rates of tumor recurrence, with Harrell's c-index of 0.746. Conclusions: Combination of preoperative MR imaging and clinical findings can be useful for predicting tumor recurrence after surgical resection of ampullary adenocarcinoma. Identifying these features before surgery may aid in better treatment planning and management of these patients. Clinical relevance statement: A predictive nomogram using preoperative MR imaging and clinical findings can be useful in estimating the recurrence-free survival after surgical resection of ampullary adenocarcinoma. Key Points: • Presently, tumor size on imaging is the only non-invasive factor that correlates with recurrence-free survival from ampullary adenocarcinoma; other factors are obtained postoperatively. • Infiltrative tumor margin, adjacent organ invasion, adjacent vessel invasion, peripancreatic lymph node enlargement on MRI, and jaundice are significant predictors for recurrence. • A nomogram incorporating significant MR imaging and clinical findings showed good performance in predicting recurrence-free survival, which can help in treatment planning. [ABSTRACT FROM AUTHOR]

Background/aims: Necrosis is an important pathological feature that reflects high malignancy potential in tumors such as hepatocellular carcinoma and renal cell carcinoma. We aimed to elucidate the prognostic impact of necrosis in ampullary carcinomas. Materials and methods: We reviewed 101 consecutive cases of ampullary carcinoma for tumor necrosis, types of necrosis, macroscopic and microscopic histopathological subtypes, lymphatic-vascular-perineural invasions, and other histopathological parameters. Results: Tumor necrosis was present in 19 (18.8%) cases and was identified as an independent poor prognostic indicator in multivariate survival analysis (p = 0.029). Conclusion: The presence of necrosis in ampullary carcinomas is directly related to vascular and perineural invasion and is a poor prognostic indicator independent of tumor stage. Including the presence of necrosis in the pathology reports of ampullary carcinomas will facilitate risk stratification. [ABSTRACT FROM AUTHOR]

Congenital anomalies of the biliary tree are rare and present challenges for endoscopists performing interventions such as endoscopic retrograde cholangiopancreatography (ERCP). The reported incidence of ectopic biliary drainage ranges from 2% to 20%, and the condition is often associated with pancreatobiliary complications, including choledocholithiasis (56%), cholangitis (39%), and acute pancreatitis (18.2%). These developmental abnormalities originate during embryogenesis. Uncommon sites for the ampullary opening include the third or fourth part of the duodenum, the stomach, and the pancreatic duct. We report a rare case of ectopic ampulla opening into the pylorus, which presented with cholangitis and was successfully managed with a modified ERCP technique.

Background/Aims: Palliative chemotherapy (PC) is not standardized for patients with advanced ampulla of Vater adenocarcinoma (AA). This multicenter, retrospective study evaluated first-line PC outcomes in patients with AA. Methods: Patients diagnosed with AA between January 2010 and December 2020 who underwent PC were enrolled from 10 institutions. Overall survival (OS) and progression-free survival (PFS) according to the chemotherapy regimen were analyzed. Results: Of 255 patients (mean age, 64.0±10.0 years; male, 57.6%), 14 (5.5%) had locally advanced AA and 241 (94.5%) had metastatic AA. Gemcitabine plus cisplatin (GP) was administered as first-line chemotherapy to 192 patients (75.3%), whereas capecitabine plus oxaliplatin (CAPOX) was administered to 39 patients (15.3%). The median OS of all patients was 19.8 months (95% confidence interval [CI], 17.3 to 22.3), and that of patients who received GP and CAPOX was 20.4 months (95% CI, 17.2 to 23.6) and 16.0 months (95% CI, 11.2 to 20.7), respectively. The median PFS of GP and CAPOX patients were 8.4 months (95% CI, 7.1 to 9.7) and 5.1 months (95% CI, 2.5 to 7.8), respectively. PC for AA demonstrated improved median outcomes in both OS and PFS compared to conventional bile duct cancers that included AA. Conclusion : s: While previous studies have shown mixed prognostic outcomes when AA was analyzed together with other biliary tract cancers, our study unveils a distinct clinical prognosis specific to AA on a large scale with systemic anticancer therapy. These findings suggest that AA is a distinct type of tumor, different from other biliary tract cancers, and AA itself could be expected to have a favorable response to PC.

Background/Aims: Endoscopic papillectomy (EP) is increasingly used as an alternative to surgery for managing benign ampullary neoplasms. However, post-EP resection margins are often positive or indeterminate, and there is no consensus on the management of ampullary adenomas with positive or indeterminate margins after EP. This study was designed to compare the long-term outcomes between resected margin-negative (RMN) and resected margin-positive/indeterminate (RMPI) groups and to identify factors associated with clinical outcomes. Methods: This retrospective analysis included patients with ampullary adenoma without evidence of adenocarcinoma who underwent EP between 2004 and 2016. The RMN and RMPI groups were compared for recurrence rates and recurrence-free duration during a mean follow-up duration of 71.7±39.8 months. Factors related to clinical outcomes were identified using multivariate analysis. Results: Of the 129 patients who underwent EP, 82 were in the RMN group and 47 were in the RMPI group. The RMPI group exhibited a higher recurrence rate compared to the RMN group (14.6% vs 34.0%, p=0.019). However, the recurrence-free duration was not significantly different between the groups (34.7±32.6 months vs 36.2±27.4 months, p=0.900). Endoscopic treatment successfully managed recurrence in both groups (75% vs 75%). Submucosal injection was a significant risk factor for residual lesions (hazard ratio, 4.11; p=0.009) and recurrence (hazard ratio, 2.57; p=0.021). Conclusion : s: Although ampullary adenomas with positive or indeterminate margins after EP showed a higher rate of recurrence at long-term follow-up, endoscopic treatment was effective with favorable long-term outcomes. Submucosal injection prior to resection was associated with increased risk of recurrence and residual lesions.

Background/Objectives: Endoscopic papillectomy (EP) is the preferred treatment for ampullary tumors because it has fewer side effects than surgical removal. This study retrospectively compared a new anchoring EP method (A-EP) with the conventional (C-EP) approach. Methods: Ninety-nine patients who underwent EP at a single medical institution between 2009 and 2021 were retrospectively reviewed. In all patients, the indications for EP were pathological adenoma with <10 mm of biliary invasion and a tumor diameter <30 mm on endoscopic ultrasonography. The exclusion criteria were antiplatelet/anticoagulant use, previous upper GI surgery, or prior biliary/pancreatic endoscopic therapy. One expert endoscopist performed the two types of EPs, A-EP and C-EP. Results: Sixty-two patients underwent A-EP, and 37 underwent C-EP. There were no significant differences in baseline characteristics, such as sex, age, tumor size, and ductal invasion on endoscopic ultrasound. The A-EP group had higher en bloc resection rates (95.2% vs. 78.4%, p = 0.010). Although the difference was not statistically significant, it tended towards fewer incidences of pancreatitis (p = 0.081) and duct stricture (p = 0.081). The recurrence rate was lower in the A-EP group (8.1% vs. 37.8%, p = 0.000). There were no significant differences between the two groups regarding the follow-up period (A-EP vs. C-EP, 725 vs. 1045 days, p = 0.109) or the days of recurrence (A-EP vs. C-EP, 341 vs. 562 days, p = 0.551). Conclusions: A-EP showed better outcomes than C-EP in terms of en bloc resection and recurrence rates, providing evidence for the efficacy of this novel EP method. [ABSTRACT FROM AUTHOR]

Simple Summary: It is generally reported that the prognostic factors for ampulla of Vater cancer include T stage, N stage, and lymphovascular invasion and subtype, and the meaning of MUC stain is controversial. This retrospective study by the authors evaluated the significance of various MUC stains in AoV cancer using single-center clinicopathological data. The results showed that MUC5AC was significant for lymph node metastasis and was furthermore valuable as a prognostic factor for predicting overall survival. If this is evaluated in addition to the hematoxylin and eosin stain, which is commonly performed in preoperative biopsy, it is expected to be a method to select groups that require more extensive LN dissection and further prevent locoregional recurrence. Introduction: Mucins play a pivotal role in epithelial carcinogenesis; however, their role remains elusive in ampulla of Vater (AoV) cancer, regardless of histological subtype. Therefore, we investigated the clinical significance of MUC1, MUC2, MUC5AC, and MUC6 expression in AoV cancer. Methods: Using samples from 68 patients with AoV cancer, we performed immunohistochemical staining for MUC1, MUC2, MUC5AC, and MUC6 using a tissue microarray. Subsequently, we analyzed their expression patterns in relation to clinicopathological parameters and patient outcomes. Results: Of the patients, 98.5% exhibited positive expression for MUC1, while MUC2, MUC5AC, and MUC6 were expressed in 44.1%, 47.1%, and 41.2% of the patients, respectively. Correlation analyses between mucin expression and clinicopathological factors revealed no significant associations, except between MUC5AC expression and N stage. Univariate analysis demonstrated significant associations between MUC5AC expression and overall survival (OS). Multivariate analysis further confirmed that MUC5AC expression was a significant predictor of OS, along with the N stage. However, MUC5AC expression was not meaningfully associated with recurrence-free survival (RFS). The patients positive for MUC5AC expression had a considerably shorter OS than those with negative expression. Conclusions: Our study provides insights into the clinical impact of mucins on AoV cancer, regardless of the histological subtype. Although MUC1 expression is universal, MUC5AC expression is a significant prognostic indicator that correlates with lymph node metastasis and poor OS. These results emphasize the possible utility of MUC5AC as a biomarker for extensive lymph node dissection and the prognostic evaluation of patients with AoV cancer. [ABSTRACT FROM AUTHOR]

Ampulla of Vater (AOV) is typically located in the second part of the duodenum. There are few reported cases of ectopic AOV over the line extending from the pylorus of the stomach down to the distal part of the duodenum. However, to the best of our knowledge, there are only five cases reported in the English literature of an ectopic AOV in the fourth part of the duodenum, with only one of them having adenocarcinoma of the ampulla. Hereby, we report the first case of ectopic AOV in the fourth part of the duodenum, presenting with obstructive due to adenocarcinoma with focal squamous differentiation. This is the case a 42-year-old lady who had a sleeve gastrectomy for morbid obesity in the past. She presented with right upper quadrant pain for one month associated with subjective fever, unintentional weight loss, pale stool, and dark urine. The physical examination revealed a deeply jaundiced lady with an unremarkable abdominal exam. A computed tomography scan of the abdomen revealed intrahepatic and extrahepatic biliary dilation with ectopic insertion of the distal CBD into the fourth part of the duodenum with no evidence of biliary stones. She underwent pancreaticoduodenectomy after difficult biliary decompression. Histopathological diagnosis was moderately differentiated adenocarcinoma, pancreaticobiliary type with focal squamous differentiation. Ectopic AOV is a very rare entity, especially when it is associated with adenosquamous carcinoma changes. [ABSTRACT FROM AUTHOR]

Abstract Background A rare case of neuroendocrine cell tumor (NET) having both conventional and mucinous components was reported. Mucinous NET is rarely encountered in the pathological diagnosis of gastrointestinal (GI) tumors. Here we examined the mechanism for transformation of conventional NETs into mucinous NETs. Case presentation: Macroscopic examination revealed a tumor with ulceration in the ampulla of Vater that measured 1.7 cm in its largest diameter. Histologically, the tumor comprised two components: a tubular/ribbon-like feature and small nests floating in a mucinous lake. The tumor nests showed sheet, nest and ribbon-like structures of small cells having eosinophilic cytoplasm as well as small-sized nuclei with dense hyperchromatin. Immunohistochemical analysis showed tumor cells positive for pan-endocrine markers (synaptophysin, CD56, INSM1 and chromogranin). Based on the histological findings, the solid and mucinous components were diagnosed as conventional and mucinous NETs, respectively. Grading was NET G2 based on 12.8% and 13.2% Ki-67-positive cells in the solid and mucinous components, respectively. Immunohistochemically, the mucin phenotype of this tumor was gastric and intestinal. Only the mucinous NET component had cytoplasmic CD10 expression. Examination using a customized gene panel detected only a DPC4 mutation, which was limited to the mucinous component. Conclusions: Coexistence of conventional and mucinous NETs could provide important insight into evaluating the NET subtype histogenesis. Moreover, molecular alterations including cytoplasmic expression of CD10 and the DPC4 mutation can contribute to interpretation of tumor pathogenesis.

Introduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life, and no cases were reported in

Background/Aims This study aimed to compare the safety of the double-guidewire technique (DGT) with that of the conventional single-guidewire technique (SGT) in real-world situations. Methods A total of 240 patients with naïve papilla who underwent endoscopic retrograde cholangiopancreatography (ERCP) at Daegu Catholic University Medical Center between January 2021 and December 2021 were included. The primary outcome was the rate of post-ERCP pancreatitis (PEP) in the SGT and DGT groups. Results A total of 163 patients (67.9%) belonged to the SGT group, and 77 (32.1%) belonged to the DGT group. The rates of successful biliary cannulation were 95.7% and 83.1% in the SGT and DGT groups, respectively (p=0.002). In the study group, PEP occurred in 14 patients (5.8%). The PEP rates were not significantly different between the SGT and DGT groups (4.3% vs. 9.1%, p=0.150). In the multivariate analysis, the age of

Adenomyomatous hyperplasia and adenomyoma are rare benign inflammatory pseudotumors of the gallbladder arising from Rokitansky-Aschoff sinuses. Occurrence of these hyperplastic conditions in the Vaterian and biliary system is extremely rare and is a concern for gastroenterologists and surgeons in distinguishing them from primary malignancies of the biliary system. Definitive diagnosis by imaging or cytopathological examination is difficult; thus, surgical resection becomes the only choice in such cases to relieve the obstruction. Here, we report two extremely rare cases of adenomyomatous hyperplasia of the extrahepatic bile duct after an extensive diagnostic workup, followed by Whipple's procedure. [ABSTRACT FROM AUTHOR]

Background: A rare case of neuroendocrine cell tumor (NET) having both conventional and mucinous components was reported. Mucinous NET is rarely encountered in the pathological diagnosis of gastrointestinal (GI) tumors. Here we examined the mechanism for transformation of conventional NETs into mucinous NETs. Case presentation: Macroscopic examination revealed a tumor with ulceration in the ampulla of Vater that measured 1.7 cm in its largest diameter. Histologically, the tumor comprised two components: a tubular/ribbon-like feature and small nests floating in a mucinous lake. The tumor nests showed sheet, nest and ribbon-like structures of small cells having eosinophilic cytoplasm as well as small-sized nuclei with dense hyperchromatin. Immunohistochemical analysis showed tumor cells positive for pan-endocrine markers (synaptophysin, CD56, INSM1 and chromogranin). Based on the histological findings, the solid and mucinous components were diagnosed as conventional and mucinous NETs, respectively. Grading was NET G2 based on 12.8% and 13.2% Ki-67-positive cells in the solid and mucinous components, respectively. Immunohistochemically, the mucin phenotype of this tumor was gastric and intestinal. Only the mucinous NET component had cytoplasmic CD10 expression. Examination using a customized gene panel detected only a DPC4 mutation, which was limited to the mucinous component. Conclusions: Coexistence of conventional and mucinous NETs could provide important insight into evaluating the NET subtype histogenesis. Moreover, molecular alterations including cytoplasmic expression of CD10 and the DPC4 mutation can contribute to interpretation of tumor pathogenesis. [ABSTRACT FROM AUTHOR]

Background: Visualization of the pancreatobiliary junction is one of the challenges faced by endoscopic ultrasonography (EUS). The water-filling technique, which allows for the observation of the ampulla at a suitable distance by injecting water into the lumen of the duodenum, was used for this purpose. However, a new gel immersion technique has recently been introduced for visualizing the gastrointestinal tract. This study investigated the effectiveness of visualizing the pancreatobiliary junction in EUS by comparing both water filling and the new gel immersion technique in identical cases. Methods: The study ran from June to December 2021. Ten images from each technique were retrospectively compared by three independent researchers. The primary result of the study was the number of images depicting the "Pancreatic and Biliary Ducts Penetrating the Duodenal Muscularis Propria" (defined as Excellent observation) in each technique. The secondary outcome was defined as gel immersion technique's safety and impact on duodenal lumen distension. Results: Ten patients used the gel immersion technique. All patients underwent the water-filling technique first, followed by gel injection after the water was completely aspirated. The average number of pictures rated as "Excellent observation," which is the primary outcome, was significantly higher with the gel immersion technique than with water filling, and no adverse events were observed. The subanalysis revealed that both convex and radial echoendoscopes are equally effective at depicting the ampulla with the gel immersion technique. Conclusions: The ability to depict the pancreatobiliary junction using the gel immersion technique is superior to that of the water-filling method, which may allow for a more detailed assessment of the ampullary region with both radial and convex echoendoscopes. This can be a useful EUS technique for diagnosing pancreaticobiliary maljunction or periampullary tumors. [ABSTRACT FROM AUTHOR]

Neuroendocrine tumors (NETs) of the ampulla of Vater are rare. Therefore, there is a lack of comprehensive information regarding their pathogenesis. We herein present the case of a patient with a 5-mm ampullary NET who demonstrated the presence of lymphatic invasion after undergoing endoscopic papillectomy. A 44-year-old woman was referred to our hospital for treatment of a grade 1 NET in the ampulla of Vater. Endoscopic ultrasonography revealed a hypoechoic mass within the submucosal layer without obvious infiltration into the common bile duct or the main pancreatic duct. We performed underwater endoscopic papillectomy (UEP) to remove the tumor with a negative margin. Pathological evaluation of the resected specimen showed a grade 1 NET with a negative margin. However, pancreaticoduodenectomy was subsequently performed because of the risk of lymph node metastasis, which was expected due to the significant number of NET cells infiltrating the endothelium of the lymphatic vessels. No lymph node metastasis or recurrence was observed during the 26-month follow-up period. UEP is a useful method to achieve complete resection for diagnostic and therapeutic purposes. UEP may be a novel option for endoscopic treatment of ampullary NET. [ABSTRACT FROM AUTHOR]

Periampullary cancer is a malignant tumor occurring around the ampullary region of the liver and pancreas, encompassing a variety of tissue types and sharing numerous biological characteristics, including interactions with the nervous system. The nervous system plays a crucial role in regulating organ development, maintaining physiological equilibrium, and ensuring life process plasticity, a role that is equally pivotal in oncology. Investigations into nervetumor interactions have unveiled their key part in controlling cancer progression, inhibiting anti-tumor immune responses, facilitating invasion and metastasis, and triggering neuropathic pain. Despite many mechanisms by which nerve fibers contribute to cancer advancement still being incompletely understood, the growing emphasis on the significance of nerves within the tumor microenvironment in recent years has set the stage for the development of groundbreaking therapies. This includes combining current neuroactive medications with established therapeutic protocols. This review centers on the mechanisms of Periampullary cancer's interactions with nerves, the influence of various types of nerve innervation on cancer evolution, and outlines the horizons for ongoing and forthcoming research. [ABSTRACT FROM AUTHOR]

Conjoining of the major pancreatic duct and common bile duct at the major duodenal papilla (MDP) is suspected to predispose cats to the clinical syndrome of "triaditis." However, microanatomy of the MDP or presence of lesions at the MDP has not been assessed in cats with or without triaditis. The aims of this study were to characterize feline MDP histomorphology and to identify associations between MDP anatomy/disease and the presence of biliary, pancreatic, or intestinal inflammation or neoplasia. Histologic assessment was prospectively performed on the MDP, duodenum, jejunum, ileum, liver, and pancreas from 124 client-owned cats undergoing postmortem examination. The majority of cats (104/124, 84%) had a complex ductular network at the MDP, with no distinction between pancreatic and common bile ducts. Lymphoid aggregates at the MDP were common (63/124, 51%). Inflammation of the MDP (MDPitis) was present in 35 of 124 cats (28%) and was often concurrent with cholangitis, pancreatitis, or enteritis (32/35, 91%), but was only associated with enteritis (19/35, 54%, P <.05). Triaditis was less common (19/124, 15%), but was associated with both conjoined MDP anatomy (19/19, 100%, P <.05) and MDPitis (12/19, 63%, P <.05). Neoplasia was present in 37 of 124 cats (29%), with lymphoma (28/37, 78%) predominating. Enteropathy-associated T-cell lymphoma type 2 (EATL2) was most common (n = 16/37, 43%) and was associated with triaditis and MDPitis (P <.05). These findings suggest that anatomy, immune activation, and/or inflammation of the MDP may play a role in the pathogenesis of triaditis. Further studies are needed to elucidate the relationships between triaditis, MDPitis, and EATL2. [ABSTRACT FROM AUTHOR]

Abstract Background The relationship between adenomyomatous hyperplasia of the Vaterian system(AV) and cancer is unclear, some reports suggest that AV is often combined with mucosal glandular dysplasia, but it is not clear whether mucosal glandular dysplasia is a risk factor for carcinogenesis of AV. The aim of this study was to retrospective analysis of role of ductal glandular dysplasia as a risk factor in the development of carcinoma in AV. Methods A total of 328 cases who underwent surgery with a final pathological diagnosis of adenomyomatous hyperplasia (AH) in the Chinese PLA General Hospital in BeiJing, China, between January 2005 and December 2021 were retrospectively collected. There were Seventeen cases(5%) in which the lesions were located in the common bile duct as well as the ampulla of Vater, and their clinical (age, sex, etc.), imaging (cholelithiasis, etc.) and pathological data (mucosal glandular dysplasia, etc.) were collected. Clinical data and pathological features of AV with or without mucosal glandular dysplasia were analyzed. Results There were 17 out of 328 cases of AH occurring in the Vaterian system (5%). Three of seventeen AV cases were associated with carcinoma (18%). Of three cases, two (12%) with the tumor lesions in the mucosal glands adjacent to the AH (biliary tract cancer and ampullary cancer), and one (6%) with carcinoma developed from AH itself in the ampulla of Vater. All carcinomas had adenomyomatous hyperplasia with nearby mucosal glandular dysplasia (MGD). The percentage of BTC or AC was higher in patients with concurrent AH and MGD compared to AH patients without MGD. The results show tendency toward statistical significance (P = 0.082). This difference was more obvious among AH with severe dysplasia compared to adenomyomatous hyperplasia with mild-moderate dysplasia (P = 0.018). Conclusion This study is the first to find that AV is associated with biliary tract cancer and ampullary cancer. In AV, the mucosal glandular dysplasia may be a risk factor for the development of malignancy. The underlying mechanism for carcinogenesis of AV could be AH itself or its secretions stimulating mucosal glands hyperplasia, then mucosal glands dysplasia. AV may be a precancerous lesion.

Background: The relationship between adenomyomatous hyperplasia of the Vaterian system(AV) and cancer is unclear, some reports suggest that AV is often combined with mucosal glandular dysplasia, but it is not clear whether mucosal glandular dysplasia is a risk factor for carcinogenesis of AV. The aim of this study was to retrospective analysis of role of ductal glandular dysplasia as a risk factor in the development of carcinoma in AV. Methods: A total of 328 cases who underwent surgery with a final pathological diagnosis of adenomyomatous hyperplasia (AH) in the Chinese PLA General Hospital in BeiJing, China, between January 2005 and December 2021 were retrospectively collected. There were Seventeen cases(5%) in which the lesions were located in the common bile duct as well as the ampulla of Vater, and their clinical (age, sex, etc.), imaging (cholelithiasis, etc.) and pathological data (mucosal glandular dysplasia, etc.) were collected. Clinical data and pathological features of AV with or without mucosal glandular dysplasia were analyzed. Results: There were 17 out of 328 cases of AH occurring in the Vaterian system (5%). Three of seventeen AV cases were associated with carcinoma (18%). Of three cases, two (12%) with the tumor lesions in the mucosal glands adjacent to the AH (biliary tract cancer and ampullary cancer), and one (6%) with carcinoma developed from AH itself in the ampulla of Vater. All carcinomas had adenomyomatous hyperplasia with nearby mucosal glandular dysplasia (MGD). The percentage of BTC or AC was higher in patients with concurrent AH and MGD compared to AH patients without MGD. The results show tendency toward statistical significance (P = 0.082). This difference was more obvious among AH with severe dysplasia compared to adenomyomatous hyperplasia with mild-moderate dysplasia (P = 0.018). Conclusion: This study is the first to find that AV is associated with biliary tract cancer and ampullary cancer. In AV, the mucosal glandular dysplasia may be a risk factor for the development of malignancy. The underlying mechanism for carcinogenesis of AV could be AH itself or its secretions stimulating mucosal glands hyperplasia, then mucosal glands dysplasia. AV may be a precancerous lesion. [ABSTRACT FROM AUTHOR]

Introduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life, and no cases were reported in <20 years of age. We report a rare case, presenting at a very young age, of well-differentiated NET involving the ampulla of Vater with lymph node metastasis. Case Presentation: An 18-year-old man presented with a 3-month history of upper abdominal pain and jaundice. Abdominal ultrasound showed a dilated common bile duct, and endoscopic retrograde cholangiopancreatography revealed two duodenal polypoid lesions, one of them overlying the ampulla of Vater, with an erythematous and ulcerated surface. Histopathological examination confirmed the diagnosis of NET grade 1. Octreotide scan revealed 2 para-aortic lymph nodes with intense radiotracer uptake. The patient had undergone Whipple surgery with para-aortic lymph node dissection. Histopathological examination of the surgical specimens was confirmatory of NET grade 2 and paraganglioma in a few of the dissected lymph nodes. Postoperatively, the patient was kept on monthly intramuscular octreotide. Follow-up gallium-68 DOTATATE is unremarkable apart from an avid left para-aortic lymph node which is showing stability over 12 months of follow-up. Conclusion: This case demonstrates that NETs of the ampulla of Vater can present at a very young age. Radical surgical excision with extended lymph node dissection and postoperative octreotide is associated with better patient outcomes and survival. [ABSTRACT FROM AUTHOR]

Background/Aims: The use of a self-expandable metal stent (SEMS) is recommended for unresectable malignant biliary obstruction (MBO). Stent-related adverse events might differ according to the position of the stent through the ampulla of Vater (AOV). We retrospectively evaluated SEMS patency and adverse events according to the position of the SEMS.Methods: In total, 280 patients who underwent endoscopic SEMS placement due to malignant distal biliary obstruction were analyzed retrospectively. Suprapapillary and transpapillary SEMS insertions were performed on 51 patients and 229 patients, respectively.Results: Between the suprapapillary group (SPG) and transpapillary group (TPG), the stent patency period was not significantly different (median [95% confidence interval]: 107 days [82.3 to 131.7] vs 120 days [99.3 to 140.7], p=0.559). There was also no significant difference in the rate of adverse events. In subgroup analysis, the stent patency for an MBO located within 2 cm from the AOV was found to be significantly shorter than that for an MBO located more than 2 cm from the AOV in the SPG (64 days [0 to 160.4] vs 127 days [82.0 to 171.9], p

Background/Aims: There are limitations in treating ampullary adenomas with intraductal extension using conventional endoscopic modalities. Endoscopic intraductal radiofrequency ablation (ID-RFA) may be useful for treating intraductal (common bile duct [CBD] and/or pancreatic duct [PD]) extensions of ampullary adenomas, but long-term data are lacking. We thus evaluated the long-term outcomes of endoscopic ID-RFA for managing ampullary adenomas with intraductal extension. Methods: Prospectively collected endoscopic ID-RFA database at Asan Medical Center was reviewed to identify consecutive patients with ampullary adenoma who underwent ID-RFA for intraductal extension between January 2018 and August 2021. Technical success, short-term and long-term clinical success, and adverse events were evaluated. Results: A total of 29 patients (14 CBD, 1 PD, and 14 CBD and PD) were analyzed. All patients had undergone endoscopic snare papillectomy prior to ID-RFA. A median of one session of ID-RFA (range, 1 to 3) for residual or relapsed intraductal extension of ampullary adenoma were successfully performed (technical success=100%). Both biliary and pancreatic stenting were routinely performed after ID-RFA to prevent ductal stricture. After a median follow-up of 776 days (interquartile range, 470 to 984 days), the short-term and long-term clinical success rates were 93% and 76%, respectively. Seven patients experienced procedural adverse events and three patients developed ductal strictures. Conclusions: Endoscopic ID-RFA showed good long-term outcomes in treating residual or relapsed ampullary adenomas with intraductal extension. Repeated ID-RFA may be considered as an option for managing recurrence. Further studies are needed to standardize the procedure.

Resection margin involvement after curative intent resection for gastric cancer results in a poor prognosis and deprives the patient of the chance for a cure. Reoperation to achieve an R0 status should guarantee tolerable morbidity and achievement of negative margins. We performed laparoscopic distal gastrectomy with extracorporeal Billroth II reconstruction in a 56-year-old woman with gastric cancer following neoadjuvant chemotherapy. Scattered cancer cells were observed in the proximal and distal resection margins on immunohistochemical staining for cytokeratin. Two weeks postoperatively, remnant total gastrectomy and supra-ampullary duodenectomy were performed. Before reoperation, percutaneous transhepatic gallbladder drainage and angiocatheter placement outside the ampulla of Vater (AoV) via the cystic duct were performed to avoid pancreaticoduodenectomy and to obtain the maximal distal margin. Duodenal transection was performed 1 cm above the AoV. The resected duodenum was 4 cm in length. The patient had no postoperative complications and received adjuvant chemotherapy 1 month after the reoperation.

Abstract Background Mixed neuroendocrine–non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater was made preoperatively. Case presentation Computed tomography revealed an enhancing periampullary tumor in a 69-year-old man with obstructive jaundice. Subsequent duodenoscopy revealed an ulcerated lesion in the swollen ampulla of Vater, from which six biopsies were collected. Pathological examination revealed adenocarcinoma in five of them. The remaining one was a neuroendocrine neoplasm according to immunohistochemical analysis. With a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy with modified Child’s reconstruction and was discharged without complications. Pathological examination revealed both adenocarcinoma and neuroendocrine carcinomas, each accounting for ≥ 30% of the tumor, resulting in a definitive diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater. Lymph node metastases with neuroendocrine components were also observed. Adjuvant chemotherapy was not administered because of the patient’s renal dysfunction. Liver and lymph node metastases were detected 2 months after surgery, the neuroendocrine component being considered responsible for that relapse. The patient underwent platinum-based chemotherapy at 50% dosage, which initially resulted in significant tumor shrinkage; however, he died 6 months after surgery. Conclusions While these tumors’ heterogeneity make definitive preoperative diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater difficult, the possibility of this disease can be considered by careful examination. Further study is needed to establish the optimal diagnostic criteria and treatment strategy.

Introduction: Ampullary neuroendocrine neoplasia (NEN) is rare and evidence regarding their management is scarce. This study aimed to describe clinicopathological features, management, and prognosis of ampullary NEN according to their endoscopic or surgical management. Methods: From a multi-institutional international database, patients treated with either endoscopic papillectomy (EP), transduodenal surgical ampullectomy (TSA), or pancreaticoduodenectomy (PD) for ampullary NEN were included. Clinical features, post-procedure complications, and recurrences were assessed. Results: 65 patients were included, 20 (30.8%) treated with EP, 19 (29.2%) with TSA, and 26 (40%) with PD. Patients were mostly asymptomatic (n = 46; 70.8%). Median tumor size was 17 mm (12–22), tumors were mostly grade 1 (70.8%) and pT2 (55.4%). Two (10%) EP resulted in severe American Society for Gastrointestinal Enterology (ASGE) adverse post-procedure complications and 10 (50%) were R0. Clavien 3–5 complications did not occur after TSA and in 4, including 1 postoperative death (15.4%) of patients after PD, with 17 (89.5%) and 26 R0 resection (100%), respectively. The pN1/2 rate was 51.9% (n = 14) after PD. Tumor size larger than 1 cm (i.e., pT stage >1) was a predictor for R1 resection (p < 0.001). Three-year overall survival and disease-free survival after EP, TSA, and PD were 92%, 68%, 92% and 92%, 85%, 73%, respectively. Conclusion: Management of ampullary NEN is challenging. EP should not be performed in lesions larger than 1 cm or with a endoscopic ultrasonography T stage beyond T1. Local resection by TSA seems safe and feasible for lesions without nodal involvement. PD should be preferred for larger ampullary NEN at risk of nodal metastasis. [ABSTRACT FROM AUTHOR]

Background. Neuroendocrine tumors are rare neoplasias accounting for 1 % of all digestive malignancies. In 2010, mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) were classified by the World Health Organization This type of tumor is extremely rare and most commonly occurs in the appendix, colon, and rectum. To date, only 30 clinical cases of MINEN of the major duodenal papilla (MDP) have been described in the available world literature, while we have not found a description of this pathology in the domestic literature.Description of the clinical case. We present a case of a 64-year-old male patient with MiNEN MDP. The first clinical manifestation of the disease was obstructive jaundice. To eliminate the jaundice, papillosphincterotomy, transpapillary stenting of the choledochus with a plastic stent was performed. MDP tumor was verifed as adenocarcinoma by histological examination. The patient underwent gastropancreatoduodenal resection. Postoperative immunohistochemical examination showed the expression of antibodies in tumor cells: Synaptophysin (clone 27G12) +++, Chromogranin A (5H7) +, CD 57 (NK-1) ++, Ki 67 (Mib 1) 80 %.Conclusion: mixed neuroendocrine (G3) non-neuroendocrine carcinoma (G2) of the major duodenal papilla with growth within the wall of the duodenum; metastases of the neuroendocrine component in three lymph nodes, adenocarcinoma metastasis in 1 out of 15 lymph nodes examined; pT2N2M0, MiNEN high grade (classified by S. La Rosa). Conclusion. Mixed neuroendocrine-non-neuroendocrine tumors of MDP are an extremely rare pathology. The accuracy of preoperative morphological diagnostics is not high, therefore, this diagnosis can be easily missed at the preoperative stage. In the morphological study of malignant tumors of MDP, a pathologist should take into account the feasibility of a combination of a neuroendocrine tumor with adenocarcinoma.

Abstract Background and Aim Obtaining endoscopic biopsies from the ampulla of Vater is important for the diagnosis of lesions that are suspicious for neoplasia. The clinical safety profile is not well defined in the literature. Our aim was to evaluate the procedure‐related readmission rate and complications from ampullary biopsy in patients undergoing duodenoscopy and endoscopic retrograde cholangiopancreatography (ERCP). Methods A retrospective data analysis was performed on adult patients at Austin Hospital who underwent ampullary biopsies between 1 January 2010 and 1 March 12022. Medical records were identified using pathology databases. The electronic health record was reviewed for baseline characteristics including demographics, date, indication for ampullary biopsy, procedure type (duodenoscopy or ERCP), and procedural associated interventions during ERCP. Readmissions to the Austin Emergency Department within 30 days following the biopsy were identified, and complications were noted. Results A total of 506 records were reviewed and 246 episodes of ampullary biopsy met the inclusion criteria. The procedure‐related readmission rate for all episodes was 6.1%, which included pain (3.3%), pancreatitis (2.0%), cholangitis (1.6%), and bleeding (0.8%). Ampullary biopsies with ERCP had a procedure‐related readmission rate of 8.4%, whereas ampullary biopsies without ERCP had a rate of 2.2%. Increased readmissions and complications were associated with male sex (P = 0.01 and P = 0.05, respectively). There was no association between the number of biopsies taken and complications. Conclusion Performing an ampullary biopsy without an associated ERCP carries a low rate of clinical complications and procedure‐related readmissions. The combination of ERCP and ampullary biopsy increases the risk four‐fold.

Background: Laparoscopic transduodenal ampullectomy (LTDA) is a functionpreserving surgery for pre-malignant tumors of the ampulla of Vater (AoV). However, it is technically challenging, and only a few case reports of LTDA are available in the literature. Methods: A total of 43 cases of pre-malignant tumors of AoV were operated in West China Hospital, Sichuan University between January 2017 and July 2022. Among these patients, 9 patients (group 1) underwent LTDA, 19 patients (group 2) underwent laparoscopic pancreaticoduodenectomy (LPD), and 15 patients (group 3) underwent open transduodenal ampullectomy (OTDA). Prospective collection and retrospective analysis of the demographic characteristics, intraoperative variables, and postoperative variables were carried out. Results: The patients in the three groups were comparable in terms of sex, age, body mass index, tumor size, and preoperative blood tests. In comparison to the patients in group 2, the patients in group 1 were found to require less operative time (159.7 ± 47.5 min vs. 298.1 ± 62.6, p < 0.01) and suffered lower blood losses (23.3 ± 16.7 ml vs. 156.8 ± 112.1, p = 0.002) and complications. Moreover, the postoperative hospital stays (POHS) were significantly shorter for patients in group 1 (9.0 ± 5.3 days vs. 15.5 ± 7.3 days, p = 0.04). Compared to patients who underwent OTDA, the patients in LTDA suffered from less blood loss. The operative time and post-operative details were comparable. Conclusion: Therefore, LTDA was found to be safe and feasible in the setting of pre-malignant tumors of AoV in well-selected patients. However, multidisciplinary preoperative planning is essential before the surgery. [ABSTRACT FROM AUTHOR]

Background: Mixed neuroendocrine–non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater was made preoperatively. Case presentation: Computed tomography revealed an enhancing periampullary tumor in a 69-year-old man with obstructive jaundice. Subsequent duodenoscopy revealed an ulcerated lesion in the swollen ampulla of Vater, from which six biopsies were collected. Pathological examination revealed adenocarcinoma in five of them. The remaining one was a neuroendocrine neoplasm according to immunohistochemical analysis. With a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy with modified Child's reconstruction and was discharged without complications. Pathological examination revealed both adenocarcinoma and neuroendocrine carcinomas, each accounting for ≥ 30% of the tumor, resulting in a definitive diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater. Lymph node metastases with neuroendocrine components were also observed. Adjuvant chemotherapy was not administered because of the patient's renal dysfunction. Liver and lymph node metastases were detected 2 months after surgery, the neuroendocrine component being considered responsible for that relapse. The patient underwent platinum-based chemotherapy at 50% dosage, which initially resulted in significant tumor shrinkage; however, he died 6 months after surgery. Conclusions: While these tumors' heterogeneity make definitive preoperative diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater difficult, the possibility of this disease can be considered by careful examination. Further study is needed to establish the optimal diagnostic criteria and treatment strategy. [ABSTRACT FROM AUTHOR]

Simple Summary: Biliary tract cancer (BTC) carries a poor prognosis. Most patients with BTC will require 'systemic' therapy, treating cancer throughout the body to control the disease and extend life while maintaining or improving the quality of life, but not to cure. There is no standard third-line systemic therapy, and few patients remain fit to receive three lines of treatment. This study assessed 97 patients from three academic centres who received three lines of palliative systemic therapy for BTC. Median survival after starting third-line treatment was 6.4 months, and from first-line treatment it was 26.9 months. The region of the biliary tract in which the cancer originated did not significantly affect prognosis. The 10 patients with an identified molecular change 'driving' the cancer, who received third-line treatment targeting that change, survived longer (12.5 months, versus 5.9 months for all other included patients). This study provides a benchmark for future clinical trials within BTC. Phase 3 trials have established standard first-line (1L) and 2L systemic therapy options for patients with advanced biliary cancer (ABC). However, a standard 3L treatment remains undefined. Clinical practice and outcomes for 3L systemic therapy in patients with ABC were therefore evaluated from three academic centres. Included patients were identified using institutional registries; demographics, staging, treatment history, and clinical outcomes were collected. Kaplan–Meier methods were used to assess progression-free survival (PFS) and overall survival (OS). Ninety-seven patients, treated between 2006 and 2022, were included; 61.9% had intrahepatic cholangiocarcinoma. At the time of analysis, there had been 91 deaths. Median PFS from initiating 3L palliative systemic therapy (mPFS3) was 3.1 months (95%CI 2.0–4.1), while mOS3 was 6.4 months (95%CI 5.5–7.3); mOS1 was 26.9 months (95%CI 23.6–30.2). Among patients with a therapy-targeted molecular aberration (10.3%; n = 10; all received in 3L), mOS3 was significantly improved versus all other included patients (12.5 vs. 5.9 months; p = 0.02). No differences in OS1 were demonstrated between anatomical subtypes. Fourth-line systemic therapy was received by 19.6% of patients (n = 19). This international multicentre analysis documents systemic therapy use in this select patient group, and provides a benchmark of outcomes for future trial design. [ABSTRACT FROM AUTHOR]

The biliary system or biliary tract is an elaborate system of coalescing channels which transports bile from the liver to the second part of the duodenum. The biliary system is divided for descriptive purposes into intrahepatic and extrahepatic parts. This article is confined to a description of the clinical and surgical anatomy of the extrahepatic biliary tract, comprising the right and left hepatic ducts, common hepatic duct, gallbladder and cystic duct and the common bile duct. Diseases of the extrahepatic biliary tract account for a considerable volume of abdominal surgical practice. These include metabolic, inflammatory, neoplastic, and congenital conditions. A detailed knowledge of the anatomy of the gallbladder and bile ducts and an awareness of the anatomical variations to which these structures are subject are essential to the conduct of safe and effective surgery involving the biliary tract, besides being of crucial importance to the accurate interpretation of radiological and ultrasound images of the extrahepatic biliary tract. [ABSTRACT FROM AUTHOR]

Background: Evidence of endoscopic papillectomy (EP) for ampullar adenoma with high-grade dysplasia (HGD) or adenocarcinoma is insufficient. Here we investigated the long-term outcomes of the advanced ampullary tumors treated by EP with careful surveillance comparing to subsequent surgery after EP. Methods: Patients treated with EP for ampullary adenoma with HGD or adenocarcinoma from the multi-center retrospective Korean cohort of ampulla of Vater tumor were categorized into EP alone versus EP with subsequent surgery groups. The overall survival (OS) and recurrence-free survival (RFS) were analyzed for unmatched and matched cohorts using propensity score with nearest neighbor method. Results: During a median 43.3 months of follow-up, 5-year OS was not significantly different between the EP alone and EP surgery groups (91.9% vs. 82.3%, P = 0.443 for unmatched cohort; 89.2% vs. 82.3%, P = 0.861 for matched cohort, respectively). Furthermore, 5-year RFS was not significantly different between the two groups (82.1% vs. 86.7%, P = 0.520 for unmatched cohort; 66.1% vs. 86.7%, P = 0.052 for matched cohort, respectively). However, the patients with positive both (lateral and deep) margins showed significantly poorer survival outcomes than those with negative margins within the EP alone group (P = 0.007). Conclusion: EP alone with careful surveillance showed comparable survival outcomes to those of EP with subsequent surgery for ampullar HGD or adenocarcinoma. Resection margin status could be a parameter to determine whether to perform subsequent radical surgery after EP. [ABSTRACT FROM AUTHOR]

Tumors of mixed neuroendocrine and nonneuroendocrine histology are classified as collision, combined, or amphicrine and can occur in most organs, including the hepato-pancreato-biliary tract. Given the rarity of mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla of Vater, the patient characteristics, management, and outcomes remain unclear. We sought to systematically review the worldwide literature on ampullary MANECs. Eligible studies were identified through a systematic search of the MEDLINE (via PubMed), Scopus, and Cochrane Library databases (end-of-search-date: January 5th, 2022), according to the PRISMA 2020 statement. A total of 39 studies reporting on 56 patients with ampullary MANEC were included. The median age was 63.0 (interquartile range [IQR]: 51.0–69.0) years and 55.6% were male (n = 25/45). Most had combined tumors (64.4%; n = 29/45), followed by collision (24.4%; n = 11/45), and amphicrine tumors (11.1%; n = 5/45). More than half had lymph node metastasis (56.8%; n = 25/44), yet only 7.9% had distant metastasis (n = 3/38). Tumor resection (i.e., mostly pancreaticoduodenectomy) was performed in 96.3% (n = 52/54), followed by adjuvant chemotherapy in 61.8% (n = 21/34). Nearly half experienced disease recurrence (47.2%; n = 17/36) over a median follow-up of 12.0 (IQR: 3.0–16.0) months, and 42.1% (n = 16/38) died over a median follow-up of 12.0 (IQR: 4.0–18.0) months. The most common cause of death was disease progression/recurrence in 81.3% (n = 13/16). Early diagnosis and management of ampullary MANEC is challenging yet crucial to improve outcomes since many patients are diagnosed at an advanced disease stage and have unfavorable outcomes. Multicenter granular data are warranted to further understand and improve outcomes in these patients. [ABSTRACT FROM AUTHOR]

Background and Aim: Obtaining endoscopic biopsies from the ampulla of Vater is important for the diagnosis of lesions that are suspicious for neoplasia. The clinical safety profile is not well defined in the literature. Our aim was to evaluate the procedure‐related readmission rate and complications from ampullary biopsy in patients undergoing duodenoscopy and endoscopic retrograde cholangiopancreatography (ERCP). Methods: A retrospective data analysis was performed on adult patients at Austin Hospital who underwent ampullary biopsies between 1 January 2010 and 1 March 12022. Medical records were identified using pathology databases. The electronic health record was reviewed for baseline characteristics including demographics, date, indication for ampullary biopsy, procedure type (duodenoscopy or ERCP), and procedural associated interventions during ERCP. Readmissions to the Austin Emergency Department within 30 days following the biopsy were identified, and complications were noted. Results: A total of 506 records were reviewed and 246 episodes of ampullary biopsy met the inclusion criteria. The procedure‐related readmission rate for all episodes was 6.1%, which included pain (3.3%), pancreatitis (2.0%), cholangitis (1.6%), and bleeding (0.8%). Ampullary biopsies with ERCP had a procedure‐related readmission rate of 8.4%, whereas ampullary biopsies without ERCP had a rate of 2.2%. Increased readmissions and complications were associated with male sex (P = 0.01 and P = 0.05, respectively). There was no association between the number of biopsies taken and complications. Conclusion: Performing an ampullary biopsy without an associated ERCP carries a low rate of clinical complications and procedure‐related readmissions. The combination of ERCP and ampullary biopsy increases the risk four‐fold. [ABSTRACT FROM AUTHOR]

Background The type of major duodenal papilla could be associated with difficult biliary cannulation at first endoscopic retrograde cholangiopancreatography (ERCP) in adults. Methods This retrospective cross-sectional study included patients undergoing ERCP for the first time by an expert endoscopist. We defined the type of papilla according to the endoscopic classification of Haraldsson in type 1-4. The outcome of interest was difficult biliary cannulation, defined according to the European Society of Gastroenterology. To assess the association of interest, we calculated crude and adjusted prevalence ratios (PRc and PRa, respectively) and their respective 95% confidence intervals (CI) using Poisson regression with robust variance models, employing bootstrap methods. For the adjusted model we included the variables age, sex, and indication for ERCP, according to an epidemiological approach. Results We included 230 patients. The most frequent type of papilla was type 1 (43.5%), and 101 (43.9%) of the patients presented difficult biliary cannulation. The results were consistent between the crude and adjusted analyses. After adjusting for age, sex, and ERCP indication, the prevalence of difficult biliary cannulation was highest in patients with papilla type 3 (PRa 3.66, 95%CI 2.49-5.84), followed by patients with papilla type 4 (PRa 3.21, 95%CI 1.82-5.75), and patients with papilla type 2 (PRa 1.95, 95%CI 1.15-3.20) compared to patients with papilla type 1. Conclusion In adults undergoing ERCP for the first time, patients with papilla type 3 had a greater prevalence of difficult biliary cannulation than patients with papilla type 1. [ABSTRACT FROM AUTHOR]

Invasive micropapillary carcinoma (IMPC) is an extremely rare and aggressive subtype of adenocarcinoma that was first described in breast cancer. It is characterised by an inside-out growth pattern with frequent lymphatic spread. Only few cases are described in the rare ampullo-pancreatobiliary location. In this case report, the diagnosis was unexpected given the patient's age and gender, as well as the negative nodal status. Despite the reported aggressive behaviour of this tumour, no optimal treatment strategy for IMPC in the ampulla of Vater has been established. In addition, this variant is not included in the recent WHO of the digestive system.

OBJECTIVES:To investigate the function of immunomarkers CK7, CK20, CK17, CDX2, MUC1, and MUC2 in the identification of primary ampullary carcinoma mixed subtype. METHODS:Forty-two cases of primary ampullary carcinoma were performed by immunohistochemical studies. The correlation between the mixed subtype and the other two subtypes and patient survival data was analyzed using the SPSS 16.0 statistical software. RESULTS:Among 42 cases, 12 (28.6%) cases were classified as mixed subtype, which showed variable expression patterns: 91.7% (11/12) for CK7, 83.3% (10/12) for CK20; 66.7% (8/12) for CK17, CDX2, and MUC1; and 50% (6/12) for MUC2. Ten (83.3%) mixed types coexpressed four or more immunomarkers. Eight (19%) intestinal subtypes mainly showed a positive expression of CK20, CDX2, and MUC2. Twenty-two (52.4%) pancreaticobiliary subtypes showed a positive expression of CK7, MUC1, and CK17. Stages III and IV diseases in mixed subtype (25%) and intestinal subtype (25%) were less than pancreaticobiliary subtype(63.6%) (p = 0.039). Follow-up data appeared to show a better survival rate for patients with mixed subtype than those with pancreaticobiliary subtypes. CONCLUSION:Immunohistochemical staining provided a more reliable means of diagnosing mixed ampulla carcinoma. Accurate subtyping of ampullary carcinoma is clinically important to select effective chemotherapy regimens and to assess disease prognosis.

Abstract We report the case of a 62‐year‐old female with a 6.3‐mm low‐grade neuroendocrine tumor of the ampulla of Vater, who underwent an endoscopic papillectomy. An endoscopic papillectomy was performed without complications. In the 26 months of follow‐up, no local recurrence or metastasis occurred. Endoscopic treatment of ampullary neuroendocrine tumors is controversial. However, endoscopic papillectomy may be considered a treatment option if neuroendocrine tumors are small (

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. [ABSTRACT FROM AUTHOR]

Malignant melanomas are aggressive cancers that can prove to be fatal, with Australia harbouring the highest incidence of skin cancers worldwide. Surprisingly, as little as 13.4% of patients undergoing surgical resection of high-risk melanomas remain disease-free after 2 years, with 31.6% showing evidence of distant spread. Although rare, secondary tumours of the ampulla of Vater have been documented, with the most common primaries involving breast, renal and melanoma cancers. We report the case of a malignant melanoma of the ampulla of Vater occurring in a patient 4-year post-surgical resection of a Stage II melanoma manifesting as acute pancreatitis with obstructive jaundice. Given the rarity of secondary ampullary tumours, metastatic melanoma should always be considered in patients with obstructive jaundice and a history of melanoma resection. In the absence of distant disease, surgery may be considered after consensus at multidisciplinary team meetings and after consideration of the patient preference. [ABSTRACT FROM AUTHOR]

Laparoscopic pancreaticoduodenectomy (LPD) has been widely adopted in institutions with sufficiently skilled practitioners. This technique requires attentive dissection around the superior mesenteric vein (SMV) and artery. Dissection around the SMV and Henle’s trunk is one of the key aspects of right hemicolectomy (RHC) ; adhesions and fibrosis around these vessels may impede LPD in patients with a history of RHC. We encountered three cases of periampullary tumors in patients with a history of RHC who were successfully treated with LPD. Cases 1, 2, and 3 were of 60-, 73-, and 74-year-old men with periampullary tumors. The operative durations in cases 1, 2, and 3 were 316, 267, and 265 min, respectively. The estimated blood loss volumes in cases 1, 2, and 3 were 20, 50, and 720 mL, respectively. The postoperative hospital stay durations in cases 1, 2, and 3 were of 13, 35, and 15 days, respectively. In conclusion, LPD following RHC may be safely completed with laparoscopy. [ABSTRACT FROM AUTHOR]

Background & Aims: Somatostatinoma of the ampulla of Vater (SAV) is a rare neuroendocrine tumor that usually appears with atypical clinical manifestations and is associated with Von Recklinghausen's disease. The aims of this study were to systematically review the literature regarding SAV and to highlight the clinicopathological characteristics and optimal therapeutic management of this rare entity. Methods: A systematic search of the literature in PubMed/Medline and Scopus databases was performed by two independent investigators, including all case reports and case series concerning SAVs from 1980 until September 2021. Results: In total, 37 articles were retrieved, including 43 patients, with a male to female ratio of 1.8:1 and a mean age of 46.8 ± 11.3 years (mean, SD). For 23 out of 43 patients (53.5%), Von Recklinghausen's disease was proved. The main clinical manifestations were abdominal pain (41.9%), jaundice (27.9%), weight loss (20.9%) and bowel disorders (20.9%). Typical histological findings included psammoma bodies, nests or clusters of epithelial cells with eosinophilic cytoplasm, while somatostatin staining was positive in 35 patients (81.4%), chromogranin-A in 21 patients (48.8%) and synaptophysin in 18 patients (41.9%). Surgery was the initial therapeutic approach in 34 patients (79.1%), whereas Whipple's procedure was the preferred surgical approach in 23 patients (53.4%). The longest survival among included patients was 13 years and only two postoperative deaths (4.7%) were reported. Conclusions: Somatostatinomas of the ampulla of Vater are rare malignancies that require increased physicians' suspicion and accurate surgical approach in order to achieve optimal therapeutic results. [ABSTRACT FROM AUTHOR]

Abstract Background Mixed neuroendocrine-non-neuroendocrine neoplasm (MINEN) is a rare disease and there is scarce literature on its diagnosis, treatment, and prognosis. We encountered two unusual cases of MINEN in the biliary tract, one in the ampulla of Vater and the other in the distal bile duct. In this report, we describe the clinical course of these two cases in detail. Case presentation Case 1: A 69-year-old woman presented with a chief complaint of epigastric pain. When endoscopic sphincterotomy and retrograde biliary drainage were performed for gallstone pancreatitis, an ulcerated lesion was found in the ampulla of the Vater. Based on the biopsy results, the lesion was diagnosed as the ampulla of Vater carcinoma and subtotal stomach-preserving pancreatoduodenectomy (SSPPD) was performed. Postoperative histopathological examination revealed the coexistence of adenocarcinoma and neuroendocrine carcinoma components, consistent with the diagnosis of MINEN. In addition, lymph node metastasis was found on the dorsal side of the pancreas and the metastatic component was adenocarcinoma. Adjuvant chemotherapy with etoposide and cisplatin was administered for 6 months, and presently the patient is alive without recurrence 64 months after surgery. Case 2: A 79-year-old man presented with a chief complaint of anorexia. Cholangiography showed severe stenosis of the distal bile duct. A biopsy was conducted from the stenotic lesion and it revealed the lesion to be adenocarcinoma. A diagnosis of distal bile duct carcinoma was made, and SSPPD was performed. Histopathological examination revealed the coexistence of adenocarcinoma and neuroendocrine carcinoma components, and the tumor was confirmed as MINEN of the distal bile duct. No adjuvant chemotherapy was administered due to the poor performance status. 7 months later, the patient was found to have a liver metastasis. Conclusion We experienced two valuable cases of biliary MINEN. To identify better treatments, it is important to consider the diversity of individual cases and to continue sharing a variety of cases with different presentations.