Prognostic value of nodal staging classification and number of examined lymph nodes among patients with ampullary cancer.

BACKGROUND: Metastatic disease in the regional lymph nodes (LNs) is a strong indicator of worse outcomes among patients after curative-intent resection of ampullary cancer (AC). This study aimed to ascertain the threshold number of examined LNs (ELNs) for AC to compare the prognosis accuracy of various nodal classification schemes relative to long-term prognosis. METHODS: Patients who underwent pancreatoduodenectomy (PD) for AC (2004-2019) were identified using the National Cancer Database. Locally weighted regression scatter plot smoothing (LOWESS) curves were used to ascertain the optimal cut point for ELNs. The accuracy of the American Joint Committee on Cancer N classification, LN ratio, and log odds transformation (LODDS) ratio to stratify patients relative to survival was examined. RESULTS: Among 8127 patients with AC, 67% were male with a median age of 67 years (IQR, 59-74). Tumors were most frequently classified as T3 (34.9%), followed by T2 (30.6%); T1 (12.9%) and T4 (17.6%) were less common. LN metastasis was identified in 4606 patients (56.7%). Among patients with nodal disease, 37.0% and 19.7% had N1 and N2 disease, respectively. The LOWESS curves identified an inflection cutoff point in the hazard of survival at 20 ELNs. The survival benefit of 20 ELNs was more pronounced among patients without LN metastasis vs patients with N1 disease (median overall survival [OS]: 54.1 months [IQR, 45.9-62.1] in ≥20 ELNs vs 39.0 months [IQR, 35.8-42.2] in <20 ELNs; P < .001) or N2 disease (median OS: 22.5 months [IQR, 18.9-26.2] in ≥20 ELNs vs 25.4 months [IQR, 23.3-27.6] in <20 ELNs; P < .001). When comparing the 4 different N classification schemes, the LODDS classification scheme yielded the highest predictive ability. CONCLUSIONS: Evaluation of a minimum of 20 LNs was needed to stratify patients with AC relative to the prognosis and to minimize stage migration. The LODDS nodal classification scheme had the highest prognostic accuracy to differentiate survival among patients after PD for AC.

Underwater endoscopic papillectomy for a small neuroendocrine tumor of the ampulla of Vater.

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.

Bile ductal mucosal dysplasia is a possible risk factor for adenocarcinoma in patients with adenomyomatous hyperplasia of the Vaterian system: a single-centre study from China.

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.

The timing of recurrence after endoscopic papillectomy.

BACKGROUND: Endoscopic papillectomy (EP) offers a safe and effective method for resection of ampullary adenomas. Data regarding the long-term resolution of adenoma following EP are limited. The aim of this study therefore was to examine the timing of recurrence after EP of ampullary adenomas. METHODS: This was a single-center retrospective study including patients who received EP for ampullary adenomas from 8/2000 to 1/2018. Patients with confirmed complete eradication of adenoma were included in the recurrence analysis with recurrence defined as finding adenomatous histology after 1 negative surveillance endoscopy. Kaplan-Meier estimates were calculated to determine recurrence rates. RESULTS: Of the 165 patients who underwent EP, 136 patients (mean age 61.9, 51.5% female) had adenomatous histology with a mean lesion size of 21.2 mm. A total of 124 (91.2%) achieved complete eradication with a follow-up of 345.8 person-years. Recurrence occurred in 20 (16.1%) patients at a mean of 3.2 (± 3) years (range 0.5-9.75 years) for a recurrence rate of 5.8 (95% CI 3.6-8.8) per 100 person-years. Nine (45%) recurrences occurred after the 1st 2 years of surveillance. Recurrence rate did not differ by baseline pathology [low-grade dysplasia: 5.2 (95% CI 3.0-9.0), high-grade dysplasia: 6.9 (95% CI 2.3-15.5), adenocarcinoma: 7.7 (95% CI 0.9-25.1)]. CONCLUSION: Recurrence remains a significant concern after EP. Given the timing of recurrence, long surveillance periods may be necessary. Larger multicenter studies are needed, however, to determine appropriate surveillance intervals.

Survival and surgical outcomes of robotic versus open pancreatoduodenectomy for ampullary cancer: A propensity score-matching comparison.

BACKGROUND/OBJECTIVE: Robotic pancreaticoduodenectomy in ampullary cancer has never been studied. This study aimed to clarify the feasibility and justification of robotic pancreaticoduodenectomy in ampullary cancer in terms of surgical risks, and oncologic and survival outcomes. METHODS: A propensity score-matching comparison of robotic and open pancreaticoduodenectomy based on seven factors commonly used to predict the survival outcomes in ampullary cancer patients. RESULTS: A total of 147 patients were enrolled, of which 101 and 46 underwent robotic and open pancreaticoduodenectomies, respectively. After propensity score-matching with a 2:1 ratio, 88 and 44 patients in the robotic and open pancreaticoduodenectomy groups were included. The operation time was of no significant difference after matching. The median intraoperative blood loss was much less in those who underwent robotic pancreaticoduodenectomy, both before (median, 120 vs. 320 c.c. P < 0.001) and after (100 vs. 335 mL P < 0.001) score-matching. There were no significant differences in terms of surgical risks, including surgical mortality, surgical morbidity, Clavien-Dindo severity classification, postoperative pancreatic fistula, delayed gastric emptying, post-pancreatectomy hemorrhage, chyle leak, bile leak, and wound infection, both before or after score-matching. The survival outcomes were also similar between the two groups, regardless of matching. CONCLUSIONS: Robotic pancreaticoduodenectomy for ampullary cancer is not only technically feasible and safe without increasing surgical risks, but also oncologically justifiable without compromising surgical radicality and survival outcomes.

Outcomes of endoscopic papillectomy of ampullary carcinoma and factors affecting additional surgery.

BACKGROUND/PURPOSE: Data on the prognosis of endoscopic papillectomy (EP) for ampullary carcinoma (AC) is limited; therefore, we aimed to identify the factors associated with endoscopically controlled AC. METHODS: Between January 2003 and October 2022, 75 patients underwent EP for ampullary tumors and were diagnosed with AC based on the pathological features of the resected tissue. The factors associated with additional surgery after EP were also evaluated. RESULTS: A total of 67 patients had ACs ranging from carcinoma in situ to tumors limited to the mucosa (M group), and eight patients had ACs ranging from those limited to the sphincter of Oddi to those invading the duodenal muscularis propria (OD group). The 3-year endoscopic tumor control (condition not requiring additional surgery) rates in the M and OD groups were 90.8% and 84.6% (p = .033), respectively. In the M group, the presence of tumor components in the resection margins was the only significant factor associated with additional surgeries (p = .010) in the univariate analysis. The 3-year endoscopic tumor control rates were 100% for negative and uncertain resection margins and 76.6% for positive margins (p = .009). CONCLUSIONS: If the AC is confined to the mucosa and the resection margins are negative or uncertain, the tumor can be well-controlled endoscopically.

Impact of extra-ampullary duodenal adenocarcinoma subtypes on surgical and oncological outcomes following pancreaticoduodenectomy.

BACKGROUND: There is little information about the relevance of extra-ampullary duodenal adenocarcinoma (EDA) subtypes. The aim of this study was to evaluate the impact of EDA subtypes on surgical and oncological outcomes following pancreatoduodenectomy (PD). METHODS: Consecutive patients undergoing PD for EDA from 2000 to 2019 were analyzed. Results were stratified by pathologic subtype (intestinal versus non-intestinal). Uni-and multivariable analyses were performed using standard statistical methods. RESULTS: The study population consisted of 70 patients, of whom 49 (70%) had an intestinal phenotype. EDA with intestinal phenotype was more frequently proximal to the Ampulla of Vater, while non-intestinal EDA was more frequently found distally (76% vs. 33%, p = 0.002). Patients with intestinal EDA were less likely to experience severe morbidity, with decreased reoperation and unplanned Intensive Care Unit admission rates relative to non-intestinal subtypes (2% vs. 29% p = 0.002, and 2% vs. 19%, p = 0.007, respectively). The median follow-up post-pancreatectomy was 73 months. Intestinal EDA was associated with improved overall and disease-free survival, with 3-year and 5-year survival rates of 71% vs. 29% and 53% vs. 24%, respectively. (p = 0.019 and p = 0.025). CONCLUSION: Intestinal-type EDA, which more often arises from supra-ampullary duodenum, was associated with better postoperative outcomes and improved survival.

Prognostic importance of margin clearance in ampullary adenocarcinoma following pancreaticoduodenectomy in a Danish population-based nationwide study.

BACKGROUND: In this Danish nationwide population-based study, we evaluated the prognostically relevant minimum tumour-free margin width following pancreaticoduodenectomy (PD) for ampullary adenocarcinoma (AAC) and evaluated whether certain margins hold independent prognostic information. METHODS: We included 128 patients who underwent PD for AAC from 2015 to 2019. Clinical and pathological data including well-known prognostic factors were retrieved from the Danish Pancreatic Cancer Database. Missing data were obtained by review of pathology reports and re-microscopy of resection specimens. All PD specimens were examined using a standardised pathological protocol including multicolour inking, axial slicing and exact reporting of margin widths. The cohort was dichotomised into involved and uninvolved groups, using different margin clearance definitions (0.5-≥3.0 mm). RESULTS: Following PD for AAC, margin clearance of ≥1 mm was independently associated with improved chance of survival compared with <1 mm (HR: 0.30, 95 % CI: 0.14-0.64 (p = 0.002)). Posterior and anterior margin widths were narrower compared with superior mesenteric artery and vein margins. Posterior margin and anterior surface had isolated prognostic significance in multivariable analysis. CONCLUSION: Following PD for AAC, margin clearance of at least 1 mm is independently associated with improved survival. Our data further indicate that anterior surface and posterior margin hold particular prognostic value.

Consensus statements on endoscopic radiofrequency ablation for malignant biliary strictures.

Endoscopy-guided endobiliary radiofrequency ablation has emerged as a novel treatment for malignant biliary strictures in recent years. When combined with biliary stenting and systemic chemotherapy, it can effectively postpone local tumor progression, improve patient's quality of life, and prolong their survival, which is mainly indicated for patients with inoperable extrahepatic cholangiocarcinoma and ampullary cancer. Based on the existing clinical evidence, the Digestive Endoscopology Branch of Chinese Medical Association, the Digestive Endoscopy Professional Committee, Endoscopic Physicians Branch of Chinese Medical Doctor Association, and the National Clinical Research Center for Digestive Diseases (Shanghai) organized relevant experts to discuss the indications, contraindications, technical operation specifications, and prevention and treatment of the complications during endoscopy-guided endobiliary radiofrequency ablation. Consensus statements were established, trying to provide references for standard treatment of malignant biliary tumors in clinical practice.

Impact of circumferential resection margin on survival in ampullary cancer: retrospective analysis.

BACKGROUND: Ampullary carcinoma is a clinically variable entity. This study aimed to evaluate prognostic factors for the outcome of resected ampullary carcinoma patients with particular intent to analyse the influence of surgical radicality. METHODS: Patients undergoing resection between 2002 and 2017 were analysed. Clinicopathological parameters, perioperative outcome and survival were examined. Risk factor analysis for postresection survival was performed. Resection margin status was evaluated according to the revised classification for pancreatic adenocarcinoma. RESULTS: A total of 234 patients were identified, 97.9 per cent (n = 229) underwent formal resection, while 2.1 per cent (n = 5) underwent ampullary resection. Histological subtypes were 46.6 per cent (n = 109) pancreatobiliary, 34.2 per cent (n = 80) intestinal, 11.5 per cent (n = 27) mixed, and 7.7 per cent (n = 18) undetermined. In the pancreatobiliary group, tumours were more advanced with more vascular resections, pT4 stage, G3 differentiation and pN+ status. Five-year overall survival was significantly different for pancreatobiliary compared to intestinal (51.7 per cent versus 72.8 per cent, P = 0.0087). In univariable analysis, age, pT4 stage, pN+, pancreatobiliary subtype and positive resection margin were significantly associated with worse overall survival. Long-term outcome was significantly better after true R0 resection (circumferential resection margin-, tumour clearance >1 mm) compared with circumferential resection margin+ (<1 mm) and R1 resections (5-year overall survival: 69.6 per cent, median overall survival 191 months versus 42.4 per cent and 53 months; P = 0.0017). CONCLUSION: Postresection survival of ampullary carcinoma patients is determined by histological subtype and surgical radicality. Intestinal differentiation is associated with less advanced tumour stages and better differentiation, which is reflected in a significantly better overall survival compared to pancreatobiliary differentiation. Despite this, true R0-resection is a prognostic key determinant in both entities, achieving 5-year survival in two-thirds of patients.

Application of single-layer continuous duct-to-mucosa pancreaticojejunostomy with two figure-of-eight sutures in total laparoscopic pancreaticoduodenectomy.

INTRODUCTION: To investigate the application potential of single-layer continuous duct-to-mucosa pancreaticojejunostomy with two figure-of-eight sutures ("1 + 2" PJ) in total laparoscopic pancreaticoduodenectomy (TLPD). Explore the advantages of "1 + 2" PJ over the traditional double-layer interrupted duct-to-mucosa pancreaticojejunostomy (traditional PJ). METHODS: We retrospectively collected the clinical data of 184 patients who were admitted in our department from Oct 2019 to Oct 2022, including 95 cases who underwent TLPD with "1 + 2" PJ and 89 cases who underwent TLPD with traditional PJ. The pre/intra/postoperation data were analyzed and compared. RESULTS: The "1 + 2" PJ procedures were successfully performed in all the 95 cases. When compared with the traditional PJ group, there were no statistically significant variations between the pre-operative and pathological data. However, the "1 + 2" PJ group had a shorter operation time (235 (210, 300) minutes vs. 310 (270, 360) minutes in the traditional PJ group, P < 0.001), shorter pancreaticojejunostomy time (15 (10, 20) minutes vs. 50 (45, 55) minutes in the traditional PJ group, P < 0.001), lower pancreatic fistula (both grade B/C) rate (4.21% vs. 12.34% in the traditional group, P = 0.044), and abdominal infection rate (2.11% vs. 8.99% in the traditional group, P = 0.044), as well as reduced hospital stay (11 (9, 15) days vs. 13 (11, 15) days in the traditional PJ group, P = 0.013). In the "1 + 2" PJ group, the median diameter of the pancreatic duct was 3 (3, 4) mm; 82 cases (86.31%) had a normal pancreatic texture, while nine (9.47%) cases had a hard texture, and seven (7.37%) cases had a soft texture; the median intraoperative blood loss was 200 (100, 400) mL and 19 cases (20.00%) needed intraoperative transfusion; eight cases (8.4%) developed postoperative complications, including four cases (4.2%) of pancreatic fistula (including both grade B/C), one case (1.1%) of bile leakage, three cases (3.2%) of delayed gastric emptying, three cases (3.2%) of postoperative hemorrhage, two cases (2.1%) of abdominal infection, and one case (1.1%) of reoperation; the median hospital stay was 13 (8, 17) days; 25 cases were pathologically classified as pancreatic cancer, 35 cases as bile duct cancer, 23 cases as duodenal cancer, and 12 cases as ampullary cancer. CONCLUSION: Single-layer continuous duct-to-mucosa pancreaticojejunostomy with two figure-of-eight sutures is a feasible and safe procedure that can be applied in TLPD.

[Treatment of Ampullary Adenocarcinoma].

The ampulla of Vater is a small projection formed by the confluence of the main pancreatic duct and common bile duct in the second part of the duodenum. Primary ampullary adenocarcinoma is a rare malignancy, accounting for only 0.2% of gastrointestinal cancers and approximately 7% of all periampullary cancers. Jaundice from a biliary obstruction is the most common symptom of ampullary adenocarcinoma. In the early stages, radical pancreatoduodenectomy is the standard surgical approach. On the other hand, no randomized controlled trial has provided evidence to guide physicians on the choice of adjuvant/palliative chemotherapy because of the rarity of the disease and the paucity of related research. This paper reports the biology, histology, current therapeutic strategies, and potential future therapies of ampullary adenocarcinoma.

Tumour origin, diagnostic accuracy and histopathological evaluation in patients with periampullary cancer: nationwide cohort study.

BACKGROUND: The prevalence of different periampullary cancers (pancreatic ductal adenocarcinoma, distal cholangiocarcinoma, ampullary cancer and duodenal cancer) is heterogeneous in the literature. During the 2010s, a standardized histopathological protocol for pancreatoduodenectomy specimens based on axial slicing was adopted in Sweden. The present study sought to provide information about periampullary cancers with regard to tumour types in curative and noncurative settings, preoperative diagnostic accuracy and the impact of a standardized evaluation of the surgical specimen on diagnosis, R status and lymph node assessment. METHODS: Data from patients diagnosed with periampullary cancer from 2010 to 2019 were retrieved from the Swedish National Registry for Pancreatic and Periampullary Cancer. RESULTS: Among non-curative patients, 3704 (83.6 per cent) were diagnosed with pancreatic ductal adenocarcinoma. Among patients treated with pancreatoduodenectomy, diagnosis was pancreatic ductal adenocarcinoma in 1380 (50.0 per cent), distal cholangiocarcinoma in 284 (10.3 per cent), ampullary cancer in 376 (13.6 per cent), duodenal cancer in 160 (5.8 per cent) and other diagnoses in 560 (20.3 per cent) patients. The preoperative diagnosis corresponded to the postoperative in 1177 (67.5 per cent) patients for pancreatic ductal adenocarcinoma, 162 (37.4 per cent) patients for distal cholangiocarcinoma, 220 (61.3 per cent) patients for ampullary cancer and 120 (53.6 per cent) patients for duodenal cancer. A higher rate of pancreatic ductal adenocarcinoma was seen in surgical specimens who underwent standardized evaluation, from 56.8 per cent to 64.3 per cent (P = 0.003). After standardization, higher rates of R1 resection (31.7 per cent versus 44.6 per cent, P < 0.001) and N1 stage (62.1 per cent versus 77.0 per cent, P < 0.001) were found. CONCLUSION: The proportion of pancreatic ductal adenocarcinoma was higher in patients in a non-curative setting compared with patients who underwent surgery. The rate of misdiagnosis for periampullary cancers was confirmed to be high. Thus, it should be taken into account when preoperative oncological treatment is considered. Standardized evaluation of the surgical specimen has increased pancreatic ductal adenocarcinoma, R1 and N1 rates.

Elevated CA 19-9 is associated with worse survival in patients with resected ampullary adenocarcinoma.

BACKGROUND: The prognostic utility of Carbohydrate Antigen 19-9 (CA 19-9) and Carcinoembryonic Antigen (CEA) in ampullary adenocarcinoma is unclear. We sought to evaluate the association between initial tumor marker levels and survival in patients with resected ampullary adenocarcinoma. METHODS: This was a single-institution, retrospective cohort study of consecutive patients who underwent pancreatoduodenectomy for ampullary adenocarcinoma from 1999 to 2021. CA 19-9 was assessed after biliary decompression. Contal and O'Quigley method determined optimal biomarker cutoff levels which were correlated with overall survival (OS) using the Kaplan-Meier method and Cox Proportional Hazards Regression. RESULTS: A total of 180 patients underwent pancreatoduodenectomy. Patients with CA 19-9 >100 U/mL had a shorter median OS (28 vs. 132 months, p < 0.001) compared to patients with CA 19-9 ≤ 100 U/mL at diagnosis. Survival was similar between pancreaticobiliary and intestinal tumor subtypes when CA 19-9 was >100 U/mL (OS:25 vs. 33 months, p = 0.415). By Cox regression analysis, CA 19-9 >100 U/mL was independently associated with worse OS (HR 2.8, p = 0.001). CONCLUSIONS: Preoperative CA 19-9 >100 U/mL was associated with shorter OS in patients with resected ampullary adenocarcinoma. CA 19-9 may be useful when counseling patients about prognosis or when considering the role of perioperative systemic therapy.

Tip-in endoscopic papillectomy for ampullary adenoma near diverticulum to minimize complications.

Toyonaga and colleagues present a novel "tip-in endoscopic papillectomy" approach for resecting ampullary tumors, aiming to minimize complications like perforation and residual tumor by adapting the colonic polyp endoscopic mucosal resection tip-in method. The technique is described with accompanying video in a case of ampullary tumor near a diverticulum.

[The first Russian experience of radiofrequency ablation in the treatment of adenoma of the major duodenal papilla with intraductal growth in the common bile duct]. / Pervyi v Rossii opyt primeneniya radiochastotnoi ablyatsii v lechenii adenomy bol'shogo sosochka dvenadtsatiperstnoi kishki s vnutriprotokovym rostom v kholedokh.

Adenoma of the major duodenal papilla can cause bile flow blockade and obstructive jaundice. We present successful endoscopic radiofrequency ablation for adenoma of the major duodenal papilla with intraductal growth in the common bile duct.

[Primary neuroendocrine tumor of the common bile duct] / Tumor neuroendocrino primario del conducto hepático común.

Primary neuroendocrine tumors (NETs) of the bile duct are extremely rare and represent only 0.2-2% of all gastrointestinal NETs. Within the biliary system, the main bile duct is the most affected site. A 28-year-old man with a 6-month history of intermittent jaundice, pruritus, and choluria. MRCP, PET-CT and endoscopic ultrasound were performed. A well-differentiated neuroendocrine neoplasia was diagnosed. Complete resection of the main bile duct was performed with lymphadenectomy of the hepatic pedicle with Roux-en-Y hepaticojejunostomy, without complications. The patient had an adequate evolution and nowadays he's disease-free. Primary neuroendocrine tumors of the bile duct are extremely rare. They may present clinically and radiologically similar to perihilar cholangiocarcinoma, which makes preoperative diagnosis difficult. Radical resection is indicated. Usually, they are well differentiated tumors, being the Ki-67 labeling index a reliable prognostic marker.

Los tumores neuroendocrinos (TNE) primarios de la vía biliar son extremadamente raros y representan sólo el 0.2-2% de todos los TNE gastrointestinales. Dentro del sistema biliar, la vía biliar principal es el sitio más afectado. Hombre de 28 años con cuadro de 6 meses de evolución caracterizado por ictericia intermitente, prurito y coluria. Se realizó colangiopancreatoresonancia magnética nuclear, PET-TC y ultrasonido endoscópico que concluyeron neoplasia neuroendocrina bien diferenciada. Se realizó resección completa de la vía biliar principal con linfadenectomía del pedículo hepático con hepaticoyeyunoanastomosis en Y de Roux, sin complicaciones. El paciente cursó adecuada evolución y se encuentra libre de enfermedad. Los tumores neuroendocrinos primarios de la vía biliar son extremadamente raros, presentándose clínica y radiológicamente como lesiones similares al colangiocarcinoma perihiliar lo que dificulta el diagnóstico preoperatorio. Está indicado su tratamiento quirúrgico radical. Suelen ser bien diferenciados, siendo el antígeno Ki-67 un marcador pronóstico confiable.

Endoscopic and Surgical Management of Non-Metastatic Ampullary Neuroendocrine Neoplasia: A Multi-Institutional Pancreas2000/EPC Study.

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.

Adenomyoma of the ampulla of Vater mimicking malignancy: A case report and literature review.

RATIONALE: Adenomyoma is a rare reactive, hamartomatous benign tumor-like lesion. Although adenomyoma can occur anywhere in the gastrointestinal tract, including the gallbladder, stomach, duodenum, and jejunum, it is very rarely observed in the extrahepatic bile duct and ampulla of Vater (AOV). The preoperative accurate diagnosis of adenomyoma of the Vaterian system, including the AOV and common bile duct, is significant to appropriate patient management. However, discriminating between benign and malignancy is highly challenging. Patients are frequently mistaken as having periampullary malignancy, thereby leading to unnecessary extensive surgical resection with a high risk of complications. PATIENT CONCERNS: A 47-year-old woman visited a local hospital owing to epigastric and right upper-quadrant abdominal pain for 2 days. DIAGNOSES: Abdominal ultrasonography performed in the local hospital revealed suspicious of a distal common bile duct malignancy. She was transferred to our hospital for further evaluation and management. INTERVENTIONS: After consulting with the patient, a multidisciplinary team, including a gastroenterologist, finally decided to perform surgery under the impression of an ampullary malignancy, and pylorus-preserving pancreatoduodenectomy was performed without any complications. She was histopathologically diagnosed with an adenomyoma of the AOV. OUTCOMES: At the 5-year follow-up, she was well and did not develop further symptoms or complications. LESSONS: Although adenomyoma is very rare, it should be included in the differential diagnosis of mass-like lesions of the AOV to avoid unnecessary surgeries.