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Pu Xi,1,&ast; Guizhong Huang,1,&ast; Kewei Huang,2,&ast; Dailei Qin,1 Zehui Yao,1 Lingmin Jiang,1 Qi Zhu,1 Chaobin He1 1Department of Pancreatobiliary Surgery, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-Sen University Cancer Center, Guangzhou, 510060, People’s Republic of China; 2State Key Laboratory of Oncology in South China, Department of Clinical Laboratory, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, 510060, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Chaobin He, Department of Pancreatobiliary Surgery, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, 651 Dongfeng Road E, Guangzhou, Guangdong, 510060, People’s Republic of China, Email hechb@sysucc.org.cnBackground: As a novel inflammatory-nutritional biomarker, the C-reactive protein–albumin–lymphocyte (CALLY) index has demonstrated significant prognostic value in various malignancies. However, research on its association with the prognosis of ampullary carcinoma (AC) is rare. This study aims to investigate the relationship between the CALLY index and the prognosis of patients with AC.Methods: We retrospectively analyzed data from 201 patients with AC at Sun Yat-sen University Cancer Center. Several clinicopathological factors and biomarkers were included in the study. Univariate and multivariate Cox regression analyses, along with competing risk analysis, were performed to identify prognostic factors for AC after pancreaticoduodenectomy (PD). Only factors with significant results in univariate analysis were included in multivariate analysis. To ensure the robustness of our findings, propensity score matching (PSM) analyses were conducted to assess survival differences according to the CALLY index.Results: The univariate and multivariate Cox regression analyses revealed that pathological type, N stage, T stage, postoperative chemotherapy regimen, and the CALLY index were all statistically significant prognostic factors for patients with AC after PD (all P values < 0.05). Taking into account non-cancer-related mortality as competing hazards, these factors remained significant predictors (all P values < 0.05). After PSM, the survival advantage observed between the low and high CALLY groups remained discernible and consistent.Conclusion: This study indicated that a reduced CALLY index correlates with a poorer cancer-specific survival in AC patients after PD, highlighting its utility as a prognostic marker for this condition.Keywords: ampullary carcinoma, prognosis, inflammation-nutritional-index, competing risk analysis, propensity matching analysis

Abstract Ampullary carcinoma (AC) of the intestinal type represents a distinct variant within the broader category of ampullary neoplasms. The scarcity of pertinent cellular models has constrained investigations centered on this particular malignancy. This research effectively generated a cell line (CL) of intestinal-type AC (DPC-X3). This newly developed CL has been continuously cultured for 1 year and has demonstrated stable passaging exceeding 60 generations. Morphologically, DPC-X3 exhibited characteristic attributes of an epithelial tumor. The cell proliferation rate of DPC-X3 exhibited a doubling interval of 79 h. Short tandem repeat (STR) analysis validated the high consistency between DPC-X3 and the patient’s primary tumor. Characteristically, DPC-X3 displayed sub diploid karyotypes, primarily featuring 44, XY inv (9), -18, -20, -22, and + mar. Under suspension culture conditions, DPC-X3 could efficiently form organoids, and DPC-X3 cells inoculated subcutaneously into NXG mice could form transplanted tumors. Drug susceptibility assays demonstrated that DPC-X3 resisted paclitaxel, oxaliplatin, 5-fluorouracil(5-FU), and gemcitabine. Immunohistochemical (IHC) evaluation revealed affirmative reactivity for CK7 and CK20 within DPC-X3 cells, while CDX2 exhibited no detectable expression. E-cadherin and Vimentin demonstrated positive immunoreactivity, whereas CEA and CA19-9 displayed faint positivity. The Ki-67 proliferation index was determined to be approximately 40%. DPC-X3 presents a valuable experimental platform for elucidating the pathogenesis of intestinal-type AC and can serve as a driver for drug development efforts.

Ampullary carcinoma (AC) of the intestinal type represents a distinct variant within the broader category of ampullary neoplasms. The scarcity of pertinent cellular models has constrained investigations centered on this particular malignancy. This research effectively generated a cell line (CL) of intestinal-type AC (DPC-X3). This newly developed CL has been continuously cultured for 1 year and has demonstrated stable passaging exceeding 60 generations. Morphologically, DPC-X3 exhibited characteristic attributes of an epithelial tumor. The cell proliferation rate of DPC-X3 exhibited a doubling interval of 79 h. Short tandem repeat (STR) analysis validated the high consistency between DPC-X3 and the patient's primary tumor. Characteristically, DPC-X3 displayed sub diploid karyotypes, primarily featuring 44, XY inv (9), -18, -20, -22, and + mar. Under suspension culture conditions, DPC-X3 could efficiently form organoids, and DPC-X3 cells inoculated subcutaneously into NXG mice could form transplanted tumors. Drug susceptibility assays demonstrated that DPC-X3 resisted paclitaxel, oxaliplatin, 5-fluorouracil(5-FU), and gemcitabine. Immunohistochemical (IHC) evaluation revealed affirmative reactivity for CK7 and CK20 within DPC-X3 cells, while CDX2 exhibited no detectable expression. E-cadherin and Vimentin demonstrated positive immunoreactivity, whereas CEA and CA19-9 displayed faint positivity. The Ki-67 proliferation index was determined to be approximately 40%. DPC-X3 presents a valuable experimental platform for elucidating the pathogenesis of intestinal-type AC and can serve as a driver for drug development efforts. [ABSTRACT FROM AUTHOR]

Neoplasms arising from the ampulla of Vater are exceedingly rare, and there is a paucity of literature regarding their diagnosis and management. Ampullary cancer typically presents with jaundice and signs of biliary obstruction. We present a case of ampullary adenocarcinoma with concomitant choledocholithiasis that proved complex and diagnostically challenging.

Background and Objectives: In ampullary cancer, 5-year survival rates are 30–50%, even with optimal resection and perioperative systemic therapies. We sought to determine the important clinicopathological features and adjuvant treatments in terms of the prognosis of patients with operable-stage ampullary carcinomas. Materials and Methods: We included 197 patients who underwent pancreaticoduodenectomy to treat ampullary carcinomas between December 2003 and May 2019. Demographics, clinical features, treatments, and outcomes/survival were analyzed. Results: The median disease-free survival (mDFS) and median overall survival (mOS) were 40.9 vs. 63.4 months, respectively. The mDFS was significantly lower in patients with lymphovascular invasion (p < 0.001) and lymph node involvement (p = 0.027). Potential predictors of decreased OS on univariate analysis included age ≥ 50 years (p = 0.045), poor performance status (p = 0.048), weight loss (p = 0.045), T3–T4 tumors (p = 0.018), surgical margin positivity (p = 0.01), lymph node involvement (p = 0.001), lymphovascular invasion (p < 0.001), perineural invasion (p = 0.007), and poor histological grade (p = 0.042). For the multivariate analysis, only nodal status (hazard ratio [HR]1.98; 95% confidence interval [CI], 1.08–3.65; p = 0.027) and surgical margin status (HR 2.61; 95% CI, 1.09–6.24; p = 0.03) were associated with OS. Conclusions: Nodal status and a positive surgical margin were independent predictors of a poor mOS for patients with ampullary carcinomas. Additional studies are required to explore the role of adjuvant therapy in patients with ampullary carcinomas. [ABSTRACT FROM AUTHOR]

Common bile duct duplications represent exceptionally rare congenital anomalies of the biliary tract. In this case report we document an unusual variant of common bile duct duplication in a 79-year-old man who underwent a pancreaticoduodenectomy for ampullary cancer. The duplication consisted of two unseparated, completely-layered, common bile ducts which originated above the cystic duct junction and terminated prior to the point of insertion into the pancreas, where the two lumens converged into a single duct. Duplication of the bile duct is rare and often goes undetected. In the present case, the anomaly was found incidentally in a patient who had a pancreaticoduodenectomy for an ampullary carcinoma. However, duplication may be associated with choledocholithiasis, cholangitis, pancreatitis, and pancreaticobiliary malignancies and it is important to be aware of the condition. [ABSTRACT FROM AUTHOR]

Abstract Pancreatic ductal adenocarcinoma (PDAC) and ampullary carcinoma (AAC) are lethal malignancies with modest benefits from surgery. SOX2 and STIM1 have been linked to anticancer activity in several human malignancies. This study included 94 tumor cases: 48 primary PDAC, 25 metastatic PDAC, and 21 primary AAC with corresponding non-tumor tissue. All cases were immunohistochemically stained for STIM1 and SOX2 and results were correlated with clinicopathologic data, patient survival, and BCL2 immunostaining results. Results revealed that STIM1 and SOX2 epithelial/stromal expressions were significantly higher in PDAC and AAC in comparison to the control groups. STIM1 and SOX2 expressions were positively correlated in the primary and metastatic PDAC (P = 0.016 and, P = 0.001, respectively). However, their expressions were not significantly associated with BCL2 expression. SOX2 epithelial/stromal expressions were positively correlated with the large tumor size in the primary AAC group (P = 0.052, P = 0.044, respectively). STIM1 stromal and SOX2 epithelial over-expressions had a bad prognostic impact on the overall survival of AAC (P = 0.002 and P = 0.001, respectively). Therefore, STIM1 and SOX2 co-expression in tumor cells and intra-tumoral stroma could contribute to the development of PDAC and AAC. STIM1/SOX2 expression is linked to a bad prognosis in AAC.

Abstract Objective To screen the risk factors affecting the recurrence risk of patients with ampullary carcinoma (AC)after radical resection, and then to construct a model for risk prediction based on Lasso-Cox regression and visualize it. Methods Clinical data were collected from 162 patients that received pancreaticoduodenectomy treatment in Hebei Provincial Cancer Hospital from January 2011 to January 2022. Lasso regression was used in the training group to screen the risk factors for recurrence. The Lasso-Cox regression and Random Survival Forest (RSF) models were compared using Delong test to determine the optimum model based on the risk factors. Finally, the selected model was validated using clinical data from the validation group. Results The patients were split into two groups, with a 7:3 ratio for training and validation. The variables screened by Lasso regression, such as CA19-9/GGT, AJCC 8th edition TNM staging, Lymph node invasion, Differentiation, Tumor size, CA19-9, Gender, GPR, PLR, Drinking history, and Complications, were used in modeling with the Lasso-Cox regression model (C-index = 0.845) and RSF model (C-index = 0.719) in the training group. According to the Delong test we chose the Lasso-Cox regression model (P = 0.019) and validated its performance with time-dependent receiver operating characteristics curves(tdROC), calibration curves, and decision curve analysis (DCA). The areas under the tdROC curves for 1, 3, and 5 years were 0.855, 0.888, and 0.924 in the training group and 0.841, 0.871, and 0.901 in the validation group, respectively. The calibration curves performed well, as well as the DCA showed higher net returns and a broader range of threshold probabilities using the predictive model. A nomogram visualization is used to display the results of the selected model. Conclusion The study established a nomogram based on the Lasso-Cox regression model for predicting recurrence in AC patients. Compared to a nomogram built via other methods, this one is more robust and accurate.

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.

Abstract A 70‐year‐old woman presented to our hospital with abdominal discomfort. Gastrointestinal endoscopy revealed an ampullary tumor, while a biopsy revealed a pathological diagnosis of adenocarcinoma. No distant metastases were observed and neoadjuvant chemotherapy and surgical resection were planned. Shortly thereafter, she developed obstructive jaundice due to the ampullary carcinoma. The patient underwent endoscopic retrograde cholangiopancreatography, during which a straight plastic stent was placed in the bile duct. The patient was discharged without complications. Neoadjuvant chemotherapy was initiated. Two months later, she was readmitted for surgery while asymptomatic. Endoscopic retrograde cholangiopancreatography was scheduled to replace the stent with a nasobiliary drainage tube for the surgery. Endoscopic imaging revealed that the proximal end of the stent had penetrated the duodenum on the oral side of the ampullary carcinoma. The distal end of the stent was grasped with forceps and the stent was successfully removed. A catheter was inserted into the bile duct orifice and cholangiography was performed, which revealed that the distal bile duct and the duodenum had formed a fistula. A guidewire was placed in the bile duct via the papilla and a nasobiliary drainage tube was placed. After endoscopic retrograde cholangiopancreatography, the patient exhibited smooth progress without issue. Pancreaticoduodenectomy was performed on the fourth day after the nasobiliary drainage tube placement, and the patient's postoperative course was uneventful. The proximal end of a biliary stent penetrating the duodenal wall is an infrequent phenomenon. This case report highlights a rare but noteworthy adverse event associated with straight biliary plastic stent placement.

Pancreatic ductal adenocarcinoma (PDAC) and ampullary carcinoma (AAC) are lethal malignancies with modest benefits from surgery. SOX2 and STIM1 have been linked to anticancer activity in several human malignancies. This study included 94 tumor cases: 48 primary PDAC, 25 metastatic PDAC, and 21 primary AAC with corresponding non-tumor tissue. All cases were immunohistochemically stained for STIM1 and SOX2 and results were correlated with clinicopathologic data, patient survival, and BCL2 immunostaining results. Results revealed that STIM1 and SOX2 epithelial/stromal expressions were significantly higher in PDAC and AAC in comparison to the control groups. STIM1 and SOX2 expressions were positively correlated in the primary and metastatic PDAC (P = 0.016 and, P = 0.001, respectively). However, their expressions were not significantly associated with BCL2 expression. SOX2 epithelial/stromal expressions were positively correlated with the large tumor size in the primary AAC group (P = 0.052, P = 0.044, respectively). STIM1 stromal and SOX2 epithelial over-expressions had a bad prognostic impact on the overall survival of AAC (P = 0.002 and P = 0.001, respectively). Therefore, STIM1 and SOX2 co-expression in tumor cells and intra-tumoral stroma could contribute to the development of PDAC and AAC. STIM1/SOX2 expression is linked to a bad prognosis in AAC. [ABSTRACT FROM AUTHOR]

Background/Purpose: There is currently no consensus on the use of endoscopic papillectomy (EP) for early stage duodenal ampullary adenocarcinoma. This study aimed to evaluate the feasibility of EP for patients with early stage duodenal ampullary adenocarcinoma. Methods: Patients who underwent EP for ampullary adenocarcinomas were investigated. Complete and clinical complete resection rates were evaluated. Clinical complete resection was defined as either complete resection or resection with positive or unknown margins but no cancer in the surgically resected specimen, or no recurrence on endoscopy after at least a 1‐year follow‐up. Results: Adenocarcinoma developed in 30 patients (carcinoma in situ [Tis]: 21, mucosal tumors [T1a(M)]: 4, tumors in the sphincter of Oddi [T1a(OD)]: 5). The complete resection rate was 60.0% (18/30) (Tis: 66.7% [14/21], T1a[M]: 50.0% [2/4], and T1a[OD]: 40.0% [2/5]). The mean follow‐up period was 46.8 months. The recurrence rate for all patients was 6.7% (2/30). The clinical complete resection rates of adenocarcinoma were 89.2% (25/28); rates for Tis, T1a(M), and T1a(OD) were 89.4% (17/19), 100% (4/4), and 80% (4/5), respectively. Conclusions: EP may potentially achieve clinical complete resection of early stage (Tis and T1a) duodenal ampullary adenocarcinomas. [ABSTRACT FROM AUTHOR]

Objective: To screen the risk factors affecting the recurrence risk of patients with ampullary carcinoma (AC)after radical resection, and then to construct a model for risk prediction based on Lasso-Cox regression and visualize it. Methods: Clinical data were collected from 162 patients that received pancreaticoduodenectomy treatment in Hebei Provincial Cancer Hospital from January 2011 to January 2022. Lasso regression was used in the training group to screen the risk factors for recurrence. The Lasso-Cox regression and Random Survival Forest (RSF) models were compared using Delong test to determine the optimum model based on the risk factors. Finally, the selected model was validated using clinical data from the validation group. Results: The patients were split into two groups, with a 7:3 ratio for training and validation. The variables screened by Lasso regression, such as CA19-9/GGT, AJCC 8th edition TNM staging, Lymph node invasion, Differentiation, Tumor size, CA19-9, Gender, GPR, PLR, Drinking history, and Complications, were used in modeling with the Lasso-Cox regression model (C-index = 0.845) and RSF model (C-index = 0.719) in the training group. According to the Delong test we chose the Lasso-Cox regression model (P = 0.019) and validated its performance with time-dependent receiver operating characteristics curves(tdROC), calibration curves, and decision curve analysis (DCA). The areas under the tdROC curves for 1, 3, and 5 years were 0.855, 0.888, and 0.924 in the training group and 0.841, 0.871, and 0.901 in the validation group, respectively. The calibration curves performed well, as well as the DCA showed higher net returns and a broader range of threshold probabilities using the predictive model. A nomogram visualization is used to display the results of the selected model. Conclusion: The study established a nomogram based on the Lasso-Cox regression model for predicting recurrence in AC patients. Compared to a nomogram built via other methods, this one is more robust and accurate. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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]

Unresectable extrahepatic cholangiocarcinoma and ampullary carcinoma have high malignant degree and poor prognosis. At present, internal or external biliary drainage is the main palliative treatment, which can improve patients' quality of life，but has no therapeutic effect on the tumor itself. Endoscopic photodynamic therapy (PDT) is a new technology for minimally invasive treatment of biliary tumors in recent years. It can kill tumor cells locally and cause systemic immune response, prolong the survival time of patients, and has a broad clinical application prospect. With the deepening of the research on the molecular mechanism of PDT and the combination with chemotherapy, radiotherapy，immunotherapy and so on, endoscopic PDT has achieved significant efficacy in killing primary tumor, reducing the tumor stage and treating the recurrent tumors. In this paper, we analyze the current status of endoscopic PDT in unresectable extrahepatic cholangiocarcinoma and ampullary carcinoma， and look forward to the future direction.

Simple Summary: Ampullary adenocarcinoma is a rare tumor in the gastrointestinal tract. Surgery is the preferred treatment, however if a patient has other medical conditions or advanced disease, surgery may not be possible. In this situation, the best treatment strategy is unknown. We sought to find out what happens to these patients in terms of treatments and survival. We used the National Cancer Database 2004–2017 to find 2176 patients who were diagnosed with ampullary adenocarcinoma but did not undergo surgery. The majority of these patients did not receive any chemotherapy or radiation. The rest received a combination of chemotherapy, palliative radiation, and/or definitive radiation. One-year overall survival ranged from 35% in patients who only received palliative radiation to 59.4% in patients who received chemotherapy and definitive radiation therapy. We did not find a significant difference in survival between patients who received chemotherapy and those who received chemotherapy and definitive radiation therapy. Surgical resection is the standard of care for ampullary adenocarcinoma (AC). Many patients are ineligible due to comorbidities/advanced disease. Evidence for the optimal non-operative management of localized AC is lacking. We hypothesize that patients treated with chemotherapy (CT) and definitive radiation (DRT) will have superior survival (OS) compared to those treated with CT alone. We performed a retrospective review of the National Cancer Database from 2004 to 2017 to identify patients with non-metastatic AC and no surgical intervention. Patients were categorized as having received no treatment, palliative radiotherapy (PRT) alone, CT alone, CT + PRT, DRT alone, or CT + DRT. We utilized Kaplan–Meier analysis to determine OS and the log-rank test to compare survival curves. Among 2176 patients, treatment groups were: No treatment (71.2%), PRT alone (1.9%), CT alone (13.1%), CT + PRT (1.6%), DRT alone (2.4%), and CT + DRT (9.7%). One-year OS varied by treatment group, ranging from 35.1% (PRT alone) to 59.4% (CT + DRT). The one-year OS in a matched cohort was not significantly different between CT alone and CT + DRT (HR 0.87, 95% CI 0.69–1.10, p = 0.87). Most patients with non-metastatic AC not treated with surgery do not receive any treatment. There is no difference in one-year OS between those undergoing CT alone and CT + DRT. [ABSTRACT FROM AUTHOR]

Background and Objectives: In ampullary cancer, 5-year survival rates are 30–50%, even with optimal resection and perioperative systemic therapies. We sought to determine the important clinicopathological features and adjuvant treatments in terms of the prognosis of patients with operable-stage ampullary carcinomas. Materials and Methods: We included 197 patients who underwent pancreaticoduodenectomy to treat ampullary carcinomas between December 2003 and May 2019. Demographics, clinical features, treatments, and outcomes/survival were analyzed. Results: The median disease-free survival (mDFS) and median overall survival (mOS) were 40.9 vs. 63.4 months, respectively. The mDFS was significantly lower in patients with lymphovascular invasion (p < 0.001) and lymph node involvement (p = 0.027). Potential predictors of decreased OS on univariate analysis included age ≥ 50 years (p = 0.045), poor performance status (p = 0.048), weight loss (p = 0.045), T3–T4 tumors (p = 0.018), surgical margin positivity (p = 0.01), lymph node involvement (p = 0.001), lymphovascular invasion (p < 0.001), perineural invasion (p = 0.007), and poor histological grade (p = 0.042). For the multivariate analysis, only nodal status (hazard ratio [HR]1.98; 95% confidence interval [CI], 1.08–3.65; p = 0.027) and surgical margin status (HR 2.61; 95% CI, 1.09–6.24; p = 0.03) were associated with OS. Conclusions: Nodal status and a positive surgical margin were independent predictors of a poor mOS for patients with ampullary carcinomas. Additional studies are required to explore the role of adjuvant therapy in patients with ampullary carcinomas.

Ampullary carcinoma is a rare malignant tumor of the digestive tract. Compared with other malignant tumors around the ampulla, its prognosis is significantly better, but the prognosis of some patients with ampulla cancer is unsatisfactory. Studies have confirmed that the systemic inflammatory response is closely related to the prognosis and efficacy of tumors, and the tumor microenvironment plays a vital role in tumor progression and prognosis. In recent years, inflammatory parameters and related prediction models have become a hot spot in predicting the prognosis and survival of ampullary cancer, and they have been confirmed to have certain predictive value. This article reviews the correlation between inflammatory parameters and prognosis of ampullary carcinoma and presents the possible progress for future research.

Background: Ampullary carcinoma (AC) is a rare cancer of the digestive system that occurs in the ampulla at the junction of the bile duct and pancreatic duct. However, there is a lack of predictive models for overall survival (OS) and disease -specific survival (DSS) in AC. This study aimed to develop a prognostic nomogram for patients with AC using data from the Surveillance, Epidemiology, and End Results Program (SEER) database. Methods: Data from 891 patients between 2004 and 2019 were downloaded and extracted from the SEER database. They were randomly divided into the development group (70%) and the verification group (30%), and then univariate and multivariate Cox proportional hazards regression, respectively, was used to explore the possible risk factors of AC. The factors significantly related to OS and DSS were used to establish the nomogram, which was assessed via the concordance index (C-index), and calibration curve. An internal validation was conducted to test the accuracy and effectiveness of the nomogram. Kaplan-Meier calculation was used to predict the further OS and DSS status of these patients. Results: On multivariate Cox proportional hazards regression, the independent prognostic risk factors associated with OS were age, surgery, chemotherapy, regional node positive (RNP), extension range and distant metastasis with a moderate C-index of 0.731 (95% confidence interval (CI): 0.719-0.744) and 0.766 (95% CI: 0.747-0.785) in the development and verification groups, respectively. While, marital status, surgery, chemotherapy, regional node positive (RNP), extension range and distant metastasis were significantly linked to AC patients' DSS, which have a better C-index of 0.756 (95% confidence interval (CI): 0.741-0.770) and 0.781 (95% CI: 0.757-0.805) in the development and verification groups. Both the survival calibration curves of 3- and 5-year OS and DSS brought out a high consistency. Conclusion: Our study yielded a satisfactory nomogram showing the survival of AC patients, which may help clinicians to assess the situation of AC patients and implement further treatment. [ABSTRACT FROM AUTHOR]

Background. The aim of this study was to evaluate symptoms, diagnostic methods, short- and long-term outcomes of surgical treatment in patients with duodenal adenocarcinoma. Patients and Methods. A single center, retrospective, observational study of 52 consecutive patients with duodenal adenocarcinoma operated on with curative intent between 2006 - 2019. Duodenectomy as part of a hemipancreatoduodenectomy or total pancreatectomy procedure was performed for ADAC (ampullary duodenal/intestinal adenocarcinoma) or NADAC (non-ampullary duodenal adenocarcinoma). Results. Prevailing symptoms were obstructive jaundice in the ADAC group (P

Background: Many studies have confirmed that diabetes was associated with prognosis in many malignant cancer types. However, the impact of diabetes on ampullary carcinoma (AC) has not been investigated. Methods: A total of 266 AC patients in the National Cancer Center of China between January 1998 and December 2020 were retrospectively reviewed. The postoperative complication rate, postoperative recurrence rate, and long-term survival were compared between the diabetes group and the no diabetes group. Results: A total of 32 AC patients (12.03%) were diagnosed with diabetes before surgery. In total, 111 patients (41.73%) had one or more postoperative complications, and there was no perioperative death. There was no statistically significant difference regarding postoperative complications between the diabetes group and the no diabetes group. Altogether, 120 patients (45.11%) experienced postoperative recurrence. Multivariate analysis revealed that diabetes was an independent risk factor for the recurrence (OR: 2.384, 95% CI: 1.065–5.336, p = 0.035), OS (HR: 1.597, 95% CI: 1.005–2.537, p = 0.047), and RFS (HR: 1.768, 95% CI: 1.068–2.925, p = 0.027) in AC patients after curative pancreatoduodenectomy. Conclusions: Diabetes may adversely affect the recurrence of patients with AC after curative pancreaticoduodenectomy, leading to an increased risk of poor prognosis in early-stage patients. Further studies involving a large sample size are needed to validate our results.

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.

Background: Cancer-associated fibroblasts (CAFs) reportedly enhance the progression of gastrointestinal surgery; however, the role of CAFs in ampullary carcinomas remains poorly examined. This study aimed to investigate the effect of CAFs on the survival of patients with ampullary carcinoma. Materials and methods: A retrospective analysis of 67 patients who underwent pancreatoduodenectomy between January 2000 and December 2021 was performed. CAFs were defined as spindle-shaped cells that expressed asmooth muscle actin (a-SMA) and fibroblast activation protein (FAP). The impact of CAFs on survival, including recurrence-free (RFS) and diseasespecific survival (DSS), as well as prognostic factors associated with survival, was analyzed. Results: The high-a-SMA group had significantly worse 5-year RFS (47.6% vs. 82.2%, p = 0.003) and 5-year DSS (67.5% vs. 93.3%, p = 0.01) than the low-a-SMA group. RFS (p = 0.04) and DSS (p = 0.02) in the high-FAP group were significantly worse than those in the low-FAP group. Multivariable analyses found that high a-SMA expression was an independent predictor of RFS [hazard ratio (HR): 3.68; 95% confidence intervals (CI): 1.21-12.4; p = 0.02] and DSS (HR: 8.54; 95% CI: 1.21-170; p = 0.03). Conclusions: CAFs, particularly a-SMA, can be useful predictors of survival in patients undergoing radical resection for ampullary carcinomas. [ABSTRACT FROM AUTHOR]

Objectives: Our aim is to investigate the prognostic value and effect on chemotherapy toxicity of pre-treatment sarcopenia determined with positron emission tomography/computerized tomography(PET/CT) in patients with ampullary carcinoma. Methods: Characteristics of patients retrieved retrospectively. Skeletal muscle area(SMA) measurement of the muscle at L3 vertebra level was evaluated for each patient from their PET/CT scan taken at the time of diagnosis. The sex-specific cut-off levels for determining sarcopenia were <38.5 cm²/m² for females and <52.4 cm²/m² for males. Association between oncological and radiological data was analyzed. Results: A total of 90 patients included in the study. Median age at diagnosis was 62(range: 44-77). Half of the patients were sarcopenic. Pre-treatment sarcopenia was determined as an independent variable predicting survival for both disease-free-survival(DFS) and overall survival(OS). Sarcopenic patients had statistically significant shorter OS(67.2 months for non-sarcopenic patients vs 53.2 months for sarcopenic patients, 95%CI:63.6-70.9, p<0.001), and a trend for shorter DFS(48 months for non-sarcopenic patients vs 36.8 months for sarcopenic patients, 95%CI:20.3-53.4, p=0.95) was also determined. On the other hand, chemotherapy related toxicity has also seen more in sarcopenic patients. Conclusion: Detecting the presence of pre-treatment sarcopenia may enable clinicians to predict the patient group with low survival and high probability of treatment toxicity. In order to protect this group of patients from toxicity, pretreatment sarcopenia measurement should be applied in routine practice and should guide treatment plan. [ABSTRACT FROM AUTHOR]

The bone tissue is a specialised connective tissue composed of several components that undergo constant remodelling. The balance between bone deposition and resorption is essential for maintaining a healthy bone structure. In case of a disruption in this remodelling process, which can lead to an imbalance between bone deposition and resorption, an increase in the opacity of a vertebral body may be observed in imaging studies, resulting in what is known as the "ivory vertebra sign". This condition can be present in many diseases such as Paget's disease, lymphomas, metastatic prostate or breast tumours and osteomyelitis. We present the case of a patient with an ivory vertebra, an uncommon radiological finding, which was related to another rare disease., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2025, Santos et al.)

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Purpose: Ampullary carcinomas (ACs) are classified as pancreatobiliary (Pb-AC), intestinal (Int-AC), or mixed (Mixed-AC). The influencing role of AC subtypes on long-term outcomes is still matter of debate. Aim of this study is to evaluate the prognostic role of the three histological variants on the overall (OS) and disease-free survival (DFS) after pancreaticoduodenectomy(PD). Methods: All PDs for AC between 2004 and 2020 were included. Patients were classified according to the histological feature in Pb-AC, Int-AC, and Mixed-AC. Five-year OS and DFS were compared among the subtypes. Additionally, the prognostic role of the histological classification on OS and DFS was evaluated. Results: Fifty-six (48.7%) Pb-ACs, 53 (46.1%) Int-ACs, and 6 (5.2%) Mixed-ACs were evaluated. A poorer 5-year OS was evidenced for the Pb-AC group (54.1%) as compared to the Int-AC cohort (80.7%) (p = 0.03), but similar to the Mixed-AC population (33%) (p = 0.45). Pb-AC presented a worse 5-year DFS (42.3%) in comparison to the Int-AC (74.8%) (p = 0.002), while no difference was evidenced in comparison to the Mixed-AC (16.7%) (p = 0.51). At the multivariate analysis, the Pb-/Mixed-AC histotype was recognized as negative prognostic factor for both OS (OR: 2.29, CI: 1.05–4.98; p = 0.04) and DFS (OR: 2.17, CI: 1–4.33; p = 0.02). Conclusion: Histological subtypes of AC play a relevant role in long-term outcomes after PD. Pb-ACs and Mixed-ACs show a more aggressive tumor biology and a consequent worse survival as compared to the Int-AC subtype. [ABSTRACT FROM AUTHOR]

Background: Many studies have confirmed that diabetes was associated with prognosis in many malignant cancer types. However, the impact of diabetes on ampullary carcinoma (AC) has not been investigated. Methods: A total of 266 AC patients in the National Cancer Center of China between January 1998 and December 2020 were retrospectively reviewed. The postoperative complication rate, postoperative recurrence rate, and long-term survival were compared between the diabetes group and the no diabetes group. Results: A total of 32 AC patients (12.03%) were diagnosed with diabetes before surgery. In total, 111 patients (41.73%) had one or more postoperative complications, and there was no perioperative death. There was no statistically significant difference regarding postoperative complications between the diabetes group and the no diabetes group. Altogether, 120 patients (45.11%) experienced postoperative recurrence. Multivariate analysis revealed that diabetes was an independent risk factor for the recurrence (OR: 2.384, 95% CI: 1.065–5.336, p = 0.035), OS (HR: 1.597, 95% CI: 1.005–2.537, p = 0.047), and RFS (HR: 1.768, 95% CI: 1.068–2.925, p = 0.027) in AC patients after curative pancreatoduodenectomy. Conclusions: Diabetes may adversely affect the recurrence of patients with AC after curative pancreaticoduodenectomy, leading to an increased risk of poor prognosis in early-stage patients. Further studies involving a large sample size are needed to validate our results. [ABSTRACT FROM AUTHOR]

Despite accounting for only 0.2 % of gastrointestinal malignancies, ampullary carcinoma is the second most common malignant tumor in the peripelvic area after pancreatic head cancer, highlighting the need for new drug delivery systems, as pancreaticoduodenectomy remains the primary treatment. In this study, we synthesized a novel Cd(II) coordination polymer (CP1), {[Me 4 N] 2 [Cd 3 (4,4′-bpdc) 4 ]} n (4,4′-H2bpdc = 4,4′-biphenyldicarboxylic acid), under hydrothermal conditions using tetramethylammonium cations as templates. The photocatalytic performance of CP1 was investigated under UV light irradiation. Hyaluronic acid (HA) and carboxymethyl chitosan (CMCS) were chemically synthesized to retain the biocompatibility of natural polysaccharides, resulting in HA/CMCS hydrogels with a three-dimensional porous structure, as revealed by scanning electron microscopy. This structure makes the hydrogel an excellent drug carrier. We recombined CP1 and the drug paclitaxel (PTX) within the hydrogel, constructing a HA/CMCS-CP1@PTX nanodrug platform. This platform exhibited excellent drug-loading performance and allowed effective temperature control through light sources, enabling controlled release rates. In vitro cell tests demonstrated that the system promoted apoptosis by modulating the expression of apoptosis-related factors and HHLA2, thereby inhibiting the progression of ampullary carcinoma. [Display omitted] • We synthesized a novel Cd(II) coordination polymer (CP1) under hydrothermal conditions using tetramethylammonium cations as templates. • The photocatalytic performance of CP1 was investigated under UV light irradiation. • Hyaluronic acid (HA) and carboxymethyl chitosan (CMCS) were chemically synthesized, resulting in HA/CMCS hydrogels with a three-dimensional porous structure. • We recombined CP1 and the drug paclitaxel (PTX) within the hydrogel, constructing a HA/CMCS-CP1@PTX nanodrug platform. • This platform exhibited excellent drug-loading performance and allowed effective temperature control through light sources, enabling controlled release rates. • In vitro cell tests demonstrated that the system promoted apoptosis by modulating the expression of apoptosis-related factors and HHLA2, thereby inhibiting the progression of ampullary carcinoma. [ABSTRACT FROM AUTHOR]

Background. The aim of this study was to evaluate symptoms, diagnostic methods, short- and long-term outcomes of surgical treatment in patients with duodenal adenocarcinoma. Patients and Methods. A single center, retrospective, observational study of 52 consecutive patients with duodenal adenocarcinoma operated on with curative intent between 2006 - 2019. Duodenectomy as part of a hemipancreatoduodenectomy or total pancreatectomy procedure was performed for ADAC (ampullary duodenal/intestinal adenocarcinoma) or NADAC (non-ampullary duodenal adenocarcinoma). Results. Prevailing symptoms were obstructive jaundice in the ADAC group (P<0.0001) and bleeding in the NADAC group (P=0.005), with larger tumor size in patients with NADAC (P=0.001). Complication rate, morbidity and mortality were comparable. Primary total pancreatoduodenectomy predominated in the NADAC group, 16.6% vs. 2.9%, and salvage completion pancreatectomy in the ADAC group, 6% vs. 0%. Significant prognostic factors for OS were perineural invasion (P=0.006) and adjuvant chemotherapy (P=0.045) in the ADAC group, and for DFS the total number of resected lymph nodes (P=0.042) and lymph node ratio (P=0.031) in the NADAC group. Median OS is 21 months and 5-year survival 27.3% in the NADAC group and 41.5 months and 52% in the ADAC group. Conclusion. Ampullary duodenal/intestinal adenocarcinomas are smaller than non-ampullary at diagnosis, with a higher rate of lymph node metastases, but with a better prognosis and long-term outcome in the presented cohort. Oral localisation of NADAC prevailed in the present cohort. Perineural invasion and postoperative oncological therapy are significant prognostic factors for OS in ADAC, but the total number of lymph nodes and lymph node ratio are significant prognostic factors for DFS in NADAC. [ABSTRACT FROM AUTHOR]

Background: Few studies have compared the oncological benefit of laparoscopic (LPD) and open pancreatoduodenectomy (OPD) for ampullary carcinoma. The aim of this study was to compare the oncological results of these two approaches. Methods: Between 2011 and 2020, 103 patients who underwent PD for ampullary carcinoma, including 31 LPD and 72 OPD, were retrospectively analyzed. Patients were matched on a 1:2 basis for age, sex, body mass index, American Society of Anaesthesiologists score, and preoperative biliary drainage. Short- and long-term outcomes of LPD and OPD were compared. Results: The 31 LPD were matched (1:2) to 62 OPD. LPD was associated with a shorter operative time (298 vs. 341 min, p = 0.02) than OPD and similar blood loss (361 vs. 341 mL, p = 0.747), but with more intra- and post-operative transfusions (29 vs. 8%, p = 0.008). There was no significant difference in postoperative mortality (6 vs. 2%), grades B/C postoperative pancreatic fistula (22 vs. 21%), delayed gastric emptying (23 vs. 35%), bleeding (22 vs. 11%), Clavien ≥ III morbidity (22 vs. 19%), or the length of hospital stay (26 vs. 21 days) between LPD and OPD, respectively, but there were more reinterventions (22 vs. 5%, p = 0.009). Pathological characteristics were similar for tumor size (21 vs. 22 mm), well differentiated tumors (41 vs. 38%), the number of harvested (23 vs. 26) or invaded lymph nodes (48 vs. 52%), R0 resection (84 vs. 90%), and other subtypes (T1/2, T3/4, phenotype). With a comparable mean follow-up (41 vs. 37 months, p = 0.59), there was no difference in 1-, 3-, and 5-year overall (p = 0.725) or recurrence-free survival (p = 0.155) which were (93, 74, 67% vs. 97, 79, 76%) and (85, 58, 58% vs. 90, 73, 73%), respectively. Conclusion: This study showed a similar long-term oncological results between LPD and OPD for ampullary carcinoma. However, the higher morbidity observed with LPD compared to OPD, restricting its use to experienced centers. [ABSTRACT FROM AUTHOR]

Simple Summary: Primary liver cancer is currently the sixth most common cancer and the third common cause of cancer-related mortality worldwide. Incidences have increased in recent year, especially in high-income countries. The epidemiology of predisposing risk factors for hepatobiliary carcinomas have changed significantly, while treatment and therapeutic options have markedly improved. Here, we provide an update of incidence, mortality, and survival trends in recent years, in Austria. While age-adjusted incidence rates remained stable in almost all hepatobiliary carcinoma subtypes—except for gall-bladder cancer—the overall survival improved significantly. Using national registries, we investigated the epidemiological trends of hepatobiliary carcinomas in Austria between 2010 and 2018 and compared them to those reported for the periods of 1990–1999 and 2000–2009. In total, 12,577 patients diagnosed with hepatocellular carcinoma (n = 7146), intrahepatic cholangiocarcinoma (n = 1858), extrahepatic cholangiocarcinoma (n = 1649), gallbladder carcinoma (n = 1365), and ampullary carcinoma (n = 559), between 2010 and 2018, were included. The median overall survival of all patients was 9.0 months. The best median overall survival was observed in patients with ampullary carcinoma (28.5 months) and the worst median overall survival was observed in patients with intrahepatic carcinoma (5.6 months). The overall survival significantly improved in all entities over the period 2010–2018 as compared with over the periods of 2000–2009 and 1990–1999. Age-adjusted incidence and mortality rates remained stable for most entities in both, men and women; only in gallbladder carcinoma, the incidence and mortality rates significantly decreased in women, whereas, in men, the incidence rates remained stable and mortality rates showed a decreasing trend. We showed that age-adjusted incidence and mortality rates were stable in most entities, except in gallbladder carcinoma. The overall survival improved in almost all entities as compared with those during 1990–2009. [ABSTRACT FROM AUTHOR]

Abstract Background Periampullary adenocarcinoma is a major clinical problem in high-risk patients including FAP population. A recent modification for visualizing the ampulla of Vater (AV) involves attaching a cap to the tip of the forward-viewing endoscope. Our aim was to compare the rates of complete visualization of AV using this cap-assisted endoscopy (CAE) approach to standard forward-viewing endoscopy (FVE). We also determined: (i) the rates of complications and additional sedation; (ii) the mean time required for duodenal examination; and (iii) the reproducibility among endoscopists performing this procedure. Methods We performed esophagogastroduodenoscopy for AV visualization in 102 > 18 years old using FVE followed by CAE. Video recordings were blinded and randomly selected for independent expert endoscopic evaluation. Results The complete visualization rate for AV was higher in CAE (97.0%) compared to FVE (51.0%) (p

Abstract Background Tuberculosis (TB) is classified according to the site of disease as pulmonary or extrapulmonary. Extrapulmonary TB is less common than its counterpart in which it can be found anywhere in the body including the liver. Similar to ampullary carcinoma, TB liver can manifest with jaundice and deranged liver function tests, particularly in the obstructed biliary systems. Case presentation A 43-year-old gentleman with locally advanced ampullary carcinoma was noticed to have multiple suspicious liver nodules intraoperatively during curative ampulla resection. The surgery was then abandoned after a biopsy. The histology was consistent with chronic granulomatous inflammation. He was then subjected to a Whipple pancreaticoduodenectomy procedure after initiation of anti-tubercular treatment. He recovered well with no evidence of tumour recurrence and worsening TB. Conclusions A high index of suspicion and quick decision making can help to diagnose a possible extrapulmonary TB masquerading as a malignant disease in a patient with curative intention of ampullary carcinoma.

Background/Aim: The optimal extent of lymph node dissection for ampullary carcinoma is controversial. The aim of this study was to investigate the efficacy of lymph node dissection for ampullary carcinoma. Patients and Methods: Between 2000 and 2020, a total of 75 patients undergoing radical resection for ampullary carcinoma were included. The efficacy index (EI) was calculated by multiplication of the frequency of lymph node metastasis (LNM) at the station and the 5-year survival rate of patients with metastasis at the station. Results: Out of 75 patients, 14 had LNM. The EI for the peri-pancreatic head (station 13 and 17) and superior mesenteric artery (station 14) lymph node were 4.4 and 3.5, respectively. Whereas the peri-gastric (station 5 and 6), common hepatic artery (station 8), and liver hilum (station 12) lymph node stations had zero EI. Although the number of patients with the station 16 dissected was small (9%), the para-aortic (station 16) lymph nodes had the highest EI of 14.3 despite being distant lymph nodes. Conclusion: We identified the distribution of LNM and survival benefit of lymph node dissection for ampullary carcinoma. Our results suggest that the optimal extent of lymph node dissection for ampullary carcinoma could be reconsidered. [ABSTRACT FROM AUTHOR]

PurposeThe aim of this study was to investigate the effect of preoperative and postoperative malnutrition on postoperative short- and long-term outcomes for ampullary carcinoma after pancreatoduodenectomy (PD).MethodsData were collected retrospectively from 511 patients with ampullary carcinoma who underwent PD between June 2012 and June 2019. Nutritional status before and at 3, 6, and 12 months after operation was assessed by the scored Patient-Generated Subjective Global Assessment (PG-SGA). The patients were classified into well-nourished, moderately malnourished, and severely malnourished group according to the PG-SGA score. Propensity score matching (PSM) was performed to adjust baseline characteristics between preoperative group A (well-nourished and moderately malnourished group) and group B (severely malnourished group). After PSM, clinicopathological variables and postoperative complications were compared between the two groups. Univariate and multivariate Cox analysis was also conducted to investigate the prognostic factors of overall survival of patients with ampullary carcinoma who underwent PD.ResultsPreoperatively, 122 (23.9%) patients were classified into well-nourished group, 189 (37.0%) into moderately malnourished group, and 200 (39.1%) into severely malnourished group. After PSM analysis, the incidence of overall postoperative complications was higher in group B than that in group A (50.5% vs. 32.5%, p < 0.001). Multivariate Cox proportional hazards regression model showed that severe malnutrition (PG-SGA score >9 scores) before operation [hazard ratio (HR) = 1.508; 95% CI, 1.103–2.061; p = 0.01] and at 6 months (HR = 4.148; 95% CI, 2.523–6.820; p < 0.001) and 12 months (HR = 5.272; 95% CI, 3.630–7.656; p < 0.001) after operation was an independent prognostic factor of patients who underwent PD for ampullary carcinoma.ConclusionsSevere malnutrition before and at 6 and 12 months after operation significantly affects the long-term survival of patients with ampullary carcinoma who underwent PD. Additionally, the preoperative malnutrition was closely related to postoperative complications.

Purpose: The aim of this study was to investigate the effect of preoperative and postoperative malnutrition on postoperative short- and long-term outcomes for ampullary carcinoma after pancreatoduodenectomy (PD). Methods: Data were collected retrospectively from 511 patients with ampullary carcinoma who underwent PD between June 2012 and June 2019. Nutritional status before and at 3, 6, and 12 months after operation was assessed by the scored Patient-Generated Subjective Global Assessment (PG-SGA). The patients were classified into well-nourished, moderately malnourished, and severely malnourished group according to the PG-SGA score. Propensity score matching (PSM) was performed to adjust baseline characteristics between preoperative group A (well-nourished and moderately malnourished group) and group B (severely malnourished group). After PSM, clinicopathological variables and postoperative complications were compared between the two groups. Univariate and multivariate Cox analysis was also conducted to investigate the prognostic factors of overall survival of patients with ampullary carcinoma who underwent PD. Results: Preoperatively, 122 (23.9%) patients were classified into well-nourished group, 189 (37.0%) into moderately malnourished group, and 200 (39.1%) into severely malnourished group. After PSM analysis, the incidence of overall postoperative complications was higher in group B than that in group A (50.5% vs. 32.5%, p < 0.001). Multivariate Cox proportional hazards regression model showed that severe malnutrition (PG-SGA score >9 scores) before operation [hazard ratio (HR) = 1.508; 95% CI, 1.103–2.061; p = 0.01] and at 6 months (HR = 4.148; 95% CI, 2.523–6.820; p < 0.001) and 12 months (HR = 5.272; 95% CI, 3.630–7.656; p < 0.001) after operation was an independent prognostic factor of patients who underwent PD for ampullary carcinoma. Conclusions: Severe malnutrition before and at 6 and 12 months after operation significantly affects the long-term survival of patients with ampullary carcinoma who underwent PD. Additionally, the preoperative malnutrition was closely related to postoperative complications. [ABSTRACT FROM AUTHOR]

Opinion Statement: ACs are rare tumors, and thus, there is a lack of prospective trials supporting treatment decisions. Moreover, although anatomically uniform, ACs comprise of biologically distinct entities, depending on what cell type they arise from. This makes the interpretation of limited data even more challenging. Overall, the clinical outcomes of patients with AC are better than those with pancreatic cancer. However, recurrence rates remain high after curative resection. Despite the absence of definitive evidence, we believe that these high recurrence rates are a rational justification for consideration of adjuvant therapy in resected disease, and therapy selection should take tumor biology, stage, resection margins, as well as patient comorbidities and performance status into account. Largely extrapolating from pancreas cancer, we recommend consideration of adjuvant chemotherapy with 6 months of dose-modified FOLFIRINOX in fit patients with pancreatobiliary subtype tumors. Alternative regimens include gemcitabine in combination with capecitabine. If chemoradiotherapy is being added, 6 weeks of radiotherapy in conjunction with 5-FU or capecitabine can be considered. For intestinal subtypes, we recommend 3-6 months of adjuvant FOLFOX. Future studies are needed to evaluate the role of contemporary, multi-agent chemotherapy and chemoradiotherapy in patients with resected and advanced ampullary adenocarcinoma. However, the logistics of performing large randomized trials in patients with a rare cancer is challenging, and the data collection, even in a carefully designed study, would likely take many years. As such, relying on data from basket trials and retrospective analysis will likely serve as guidance for treatment decisions in the near future. Treatment of metastatic disease should employ regimens that are typically used to treat pancreas cancer for tumors of pancreatobiliary subtype and 5-FU-based regimens for intestinal subtypes. Studies specific for patients with advanced AC are much needed. Molecular testing using next-generation sequencing and testing for microsatellite instability (MSI) should be performed on all tumors. We now have disease agnostic options based on these results. Pembrolizumab is approved for MSI-H tumors and tumors with high tumor mutational burden regardless of the primary site. Larotrectinib is approved for tumors with NTRK fusions. At a time when numerous therapeutic agents are in development, for example, those targeting specific K-RAS alterations or NRG fusions, identifying molecular aberrations can significantly impact patient outcomes as well as provide further insights into the biology of disease. In addition, based on recent data suggesting a significant prevalence of germline alterations in patients with ampullary tumors, referral to genetics counselors and germline testing is warranted in a significant proportion of patients with AC. [ABSTRACT FROM AUTHOR]

Background: Orbital metastasis from ampullary carcinoma is rare, with no previously reported cases. Case presentation: We report the case of a 60-year-old man who complained of a right-sided headache, blurred vision, progressive proptosis, ptosis, and right eye pain for 3 months. His past medical history included an ampullary adenocarcinoma stage IIIA treated via the Whipple procedure and adjuvant chemoradiotherapy 1 year ago. However, he was lost to follow-up. Computed tomography of the orbit showed a soft tissue lesion in the right orbital fossa measuring 3.3 × 2 × 2 cm. An orbital mass biopsy demonstrated an intestinal-type adenocarcinoma that tested positive for cytokeratins 7 and 20 and CDX2 on immunohistochemical staining. The pathologic diagnosis was metastatic adenocarcinoma from the ampulla of Vater. Despite oncological treatment, the patient’s illness progressed. He received palliative treatment and died 1 month later. Conclusions: We presented a rare case of orbital metastasis from ampullary adenocarcinoma. This should be considered in the differential diagnosis of patients with a history of ampullary adenocarcinoma who present with symptoms referring to the relevant locations. [ABSTRACT FROM AUTHOR]

Abstract Background Clinical evidence of intraductal dissemination through the pancreatic duct has been rare. We herein describe a case of ampullary carcinoma that disseminated in the remnant pancreas through the pancreatic duct. Case presentation A 68-year-old woman underwent SSPPD for ampullary carcinoma. The tumor was diagnosed as adenocarcinoma without lymph node metastasis (T2N0M0, stage IB). Computed tomography (CT) performed 3 years later revealed a 14-mm tumor near the site of the pancreaticojejunal anastomosis. Endoscopic ultrasound-guided fine needle aspiration showed adenocarcinoma that was morphologically similar to the specimen from the first surgery. We diagnosed recurrence of ampullary carcinoma in the remnant pancreas. A total remnant pancreatectomy was performed. We found a white solid tumor at the 20-mm distal side of pancreaticojejunal anastomosis. The tumor was morphologically similar and immunostaining showed a pattern identical to that of the original tumor, suggesting that the two tumors were of the same origin. Conclusion The recurrent lesion was most likely the result of tumor cells leaving the tumor and implanting in the remnant pancreatic duct epithelium. Intraductal dissemination of adenocarcinoma is thought to be a cause of remnant recurrence after SSPPD in cases of obstruction of the pancreatic duct or an iatrogenic procedure.