Materiały źródłowe

• #1 Demographics, tumor characteristics, treatment, and clinical outcomes of patients with ampullary cancer: a Surveillance, Epidemiology, and End Results (SEER) cohort study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/30488680/

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers. The objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with ampullary tumors. […] Data on ampullary cancer between 2004 and 2013 was extracted from the Surveillance, Epidemiology and End Results (SEER) Registry. The clinical epidemiology of these tumors was analyzed using SEER*Stat. […] A total of 6803 patients with ampullary cancer were identified. Median age at diagnosis was 7113 years. The overall age-adjusted incidence of ampullary cancer was 0.59 per 100,000 per year. A higher incidence of ampullary cancer was observed in males compared to females (0.74 vs. 0.48 per 100,000 per year). […] The annual incidence of ampullary cancer has been fairly constant, though males are more likely to be affected. While its incidence increases with age, patients who are treated by surgical intervention have significantly better outcomes. Additionally, through the use of endoscopic techniques, ampullary cancer can be detected and treated much earlier.

• #2

  https://journals.lww.com/ajg/fulltext/2018/10001/demographics,_tumor_characteristics,_treatment,.85.aspx

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers. […] The overall age-adjusted incidence of ampullary cancer between 2004 and 2014 was 0.59 per 100,000 per year. […] A constant incidence rate was noted during the study period; however, a higher incidence of ampullary tumors was observed in males compared to females (0.74 vs 0.48 per 100,000 per year). […] One and 5-year cause-specific survival for ampullary cancer was 71.7% and 38.8 respectively, with a median survival of 31 months. […] Ampullary cancer is rare and has a low survival rate. Its incidence increases with age, however, those treated surgically tend to have better outcomes.

• #3 Epidemiology, clinical presentation, diagnosis, and staging of ampullary carcinoma – UpToDate

  https://www.uptodate.com/contents/ampullary-carcinoma-epidemiology-clinical-manifestations-diagnosis-and-staging

  Epidemiology, clinical features, diagnosis, and staging of ampullary carcinoma will be reviewed here. […] Neoplastic transformation of the intestinal mucosa occurs more commonly near the ampulla than at any other site in the small intestine. Despite this, primary ampullary tumors are rare, with an incidence of approximately 4 to 10 cases per million population. They account for only 6 percent of lesions that arise in the periampullary region but are responsible for 20 percent of all tumor-related obstructions of the common bile duct. There is some evidence that the incidence has increased over the last 30 years, although this has not been seen in all studies. […] Both benign and malignant ampullary tumors can occur sporadically or in the setting of a genetic syndrome. The incidence of ampullary tumors is increased 200- to 300-fold among patients with hereditary polyposis syndromes, such as familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome), compared with the general population. Surveillance endoscopy is particularly important to detect early ampullary lesions in patients with FAP given the high incidence of coexisting premalignant duodenal adenomatous polyps. Up to 90 percent of patients with FAP develop adenomas in the upper gastrointestinal tract.

• #4 Ampullary cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/ampullary-cancer

  Ampullary cancer is very rare. In the UK, around 550 people are diagnosed with ampullary cancer each year. […] These statistics are based on the average annual number of new cases of ampullary cancer (ICD10 C24.1) in the UK between 2017 and 2019.

• #5 Epidemiology, clinical presentation, diagnosis, and staging of ampullary carcinoma – UpToDate

  https://www.uptodate.com/contents/ampullary-carcinoma-epidemiology-clinical-manifestations-diagnosis-and-staging

  Epidemiology, clinical features, diagnosis, and staging of ampullary carcinoma will be reviewed here. […] Neoplastic transformation of the intestinal mucosa occurs more commonly near the ampulla than at any other site in the small intestine. Despite this, primary ampullary tumors are rare, with an incidence of approximately 4 to 10 cases per million population. They account for only 6 percent of lesions that arise in the periampullary region but are responsible for 20 percent of all tumor-related obstructions of the common bile duct. There is some evidence that the incidence has increased over the last 30 years, although this has not been seen in all studies. […] Both benign and malignant ampullary tumors can occur sporadically or in the setting of a genetic syndrome. The incidence of ampullary tumors is increased 200- to 300-fold among patients with hereditary polyposis syndromes, such as familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome), compared with the general population. Surveillance endoscopy is particularly important to detect early ampullary lesions in patients with FAP given the high incidence of coexisting premalignant duodenal adenomatous polyps. Up to 90 percent of patients with FAP develop adenomas in the upper gastrointestinal tract.

• #6 Ampullary Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276413-overview

  Ampullary carcinoma is a relatively uncommon tumor that accounts for approximately 0.2% of gastrointestinal tract malignancies and approximately 7% of all periampullary carcinomas in the United States. A review of data from the National Cancer Institutes Surveillance, Epidemiology and End Results (SEER) program found 6803 patients with ampullary cancer between 2004 and 2013; the annual incidence has been fairly constant since 2004. […] An epidemiologic study of ampullary cancer in the Netherlands found that the incidence rate increased from 0.59 per 100,000 in 1989-1995 to 0.68 per 100,000 in 2010-2016. In patients with non-metastatic ampullary disease, five-year overall survival increased from 19.8% in 1989-1995 to 29.1% in 2010-2016. […] Because ampullary carcinoma is relatively uncommon, studies of the patterns of occurrence among different ethnic groups have not been conducted. Ampullary cancer is more common in men, according to the SEER program.

• #7 Molecular Targets and Therapies for Ampullary Cancer in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 2D (2024)

  https://jnccn.org/abstract/journals/jnccn/22/2D/article-e237051.xml

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers, but incidence has been increasing since 1970 at a rate of 0.5% to 0.9% per year. It remains uncertain how improved imaging and procedural modalities have contributed to this increase. Multiple meta-analyses of ampullary, hepatic, pancreatic, and biliary cancers demonstrate that most patients are male and aged 60 years, with an increasing incidence over the previous 3 decades. Black race, higher stage and grade, and nonsurgical treatment are associated with worsened clinical outcomes. […] Ampullary carcinomas are rare but increasing in incidence. Ampullary cancers have molecular alterations that guide choice of therapy, particularly in nonresectable cases. These alterations can be more common by subtype (intestinal, pancreaticobiliary, or mixed), and next-generation sequencing is recommended for all patients who cannot undergo surgery.

• #8 Updates in Management of Ampullary Carcinomas

  https://www.primescholars.com/articles/updates-in-management-of-ampullary-carcinomas-97508.html

  Ampullary carcinomas are rare malignancies representing less than 1% of all gastrointestinal cancers. […] However, the incidence rate of ampullary cancers has increased by about 1% every year over the past 30 years. […] The standard treatment for ampullary carcinoma has been complete surgical resection via pancreaticoduodenectomy. […] Evidence suggests that pancreaticoduodenectomy has significantly improved survival rates in ampullary carcinomas with 5-year survival rates up to 39%. […] A recent prospective trial in 2010 analyzed survival of 450 patients with ampullary tumors who underwent pancreaticoduodenectomy and found that the 5-year survival rate was 45%. […] There has been a lack of specific therapeutic protocols for adjuvant treatment of ampullary cancer. […] A retrospective study from Narang et al. compared surgery plus adjuvant chemoradiation with surgery alone for patients with ampullary cancer.

• #9 Molecular Targets and Therapies for Ampullary Cancer in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 2D (2024)

  https://jnccn.org/abstract/journals/jnccn/22/2D/article-e237051.xml

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers, but incidence has been increasing since 1970 at a rate of 0.5% to 0.9% per year. It remains uncertain how improved imaging and procedural modalities have contributed to this increase. Multiple meta-analyses of ampullary, hepatic, pancreatic, and biliary cancers demonstrate that most patients are male and aged 60 years, with an increasing incidence over the previous 3 decades. Black race, higher stage and grade, and nonsurgical treatment are associated with worsened clinical outcomes. […] Ampullary carcinomas are rare but increasing in incidence. Ampullary cancers have molecular alterations that guide choice of therapy, particularly in nonresectable cases. These alterations can be more common by subtype (intestinal, pancreaticobiliary, or mixed), and next-generation sequencing is recommended for all patients who cannot undergo surgery.

• #10 Demographics, tumor characteristics, treatment, and clinical outcomes of patients with ampullary cancer: a Surveillance, Epidemiology, and End Results (SEER) cohort study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/30488680/

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers. The objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with ampullary tumors. […] Data on ampullary cancer between 2004 and 2013 was extracted from the Surveillance, Epidemiology and End Results (SEER) Registry. The clinical epidemiology of these tumors was analyzed using SEER*Stat. […] A total of 6803 patients with ampullary cancer were identified. Median age at diagnosis was 7113 years. The overall age-adjusted incidence of ampullary cancer was 0.59 per 100,000 per year. A higher incidence of ampullary cancer was observed in males compared to females (0.74 vs. 0.48 per 100,000 per year). […] The annual incidence of ampullary cancer has been fairly constant, though males are more likely to be affected. While its incidence increases with age, patients who are treated by surgical intervention have significantly better outcomes. Additionally, through the use of endoscopic techniques, ampullary cancer can be detected and treated much earlier.

• #11 Demographics, tumor characteristics, treatment, and clinical outcomes of patients with ampullary cancer: a Surveillance, Epidemiology, and End Results (SEER) cohort study – Minerva Gastroenterologica e Dietologica 2019 June;65(2):85-90 – Minerva Medica –

  https://www.minervamedica.it/en/journals/gastroenterology/article.php?cod=R08Y2019N02A0085

  BACKGROUND: Ampullary cancer accounts for only 0.2% of gastrointestinal cancers. […] The overall age-adjusted incidence of ampullary cancer was 0.59 per 100,000 per year. A higher incidence of ampullary cancer was observed in males compared to females (0.74 vs. 0.48 per 100,000 per year). […] The annual incidence of ampullary cancer has been fairly constant, though males are more likely to be affected. While its incidence increases with age, patients who are treated by surgical intervention have significantly better outcomes. Additionally, through the use of endoscopic techniques, ampullary cancer can be detected and treated much earlier.

• #12 Ampullary adenocarcinoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/ampullary-adenocarcinoma?lang=us

  These are rare tumors, with an adulthood estimated incidence of 0.7 in 100,000 men and 0.45 in 100,000 women in the United States 2. […] There is an association with Gardner syndrome.

• #13 Ampullary adenocarcinoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/ampullary-adenocarcinoma?embed_domain=hackmd.io%2F%40yIPUAFeCSL2JsU8smR5nJQ%2Fbnjhjgjghjghjgh&lang=us

  Ampullary adenocarcinomas are rare biliary tumors arising from the distal biliary epithelium at the ampulla of Vater. […] These are rare tumors, with an adulthood estimated incidence of 0.7 in 100,000 men and 0.45 in 100,000 women in the United States. […] There is an association with Gardner syndrome.

• #14 Demographics, tumor characteristics, treatment, and clinical outcomes of patients with ampullary cancer: a Surveillance, Epidemiology, and End Results (SEER) cohort study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/30488680/

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers. The objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with ampullary tumors. […] Data on ampullary cancer between 2004 and 2013 was extracted from the Surveillance, Epidemiology and End Results (SEER) Registry. The clinical epidemiology of these tumors was analyzed using SEER*Stat. […] A total of 6803 patients with ampullary cancer were identified. Median age at diagnosis was 7113 years. The overall age-adjusted incidence of ampullary cancer was 0.59 per 100,000 per year. A higher incidence of ampullary cancer was observed in males compared to females (0.74 vs. 0.48 per 100,000 per year). […] The annual incidence of ampullary cancer has been fairly constant, though males are more likely to be affected. While its incidence increases with age, patients who are treated by surgical intervention have significantly better outcomes. Additionally, through the use of endoscopic techniques, ampullary cancer can be detected and treated much earlier.

• #15 Carcinoma of the Ampulla of Vater: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/282920-overview

  Carcinoma of the ampulla of Vater is an uncommon tumor. Between 1973 and 2005, 5,625 cases were recorded in the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute. Ampullary cancer accounts for approximately 0.5% of all gastrointestinal tract malignancies. The incidence has been increasing since 1973 at an annual percentage rate of 0.9%. Prior to the recently reported increase, the quoted incidence for ampullary carcinoma was 0.2% of all gastrointestinal malignancies and 6% of all periampullary tumors. […] Most of these tumors are resectable for cure at diagnosis; however, the 5-year survival rate is only approximately 40% to 67% at best. […] A higher rate of ampullary cancer is observed in men. Ampullary cancer is most often diagnosed in the fifth through the seventh decades of life.

• #16 Ampullary carcinoma | PPT

  https://www.slideshare.net/slideshow/ampullary-carcinoma/38417037

  This document discusses ampullary carcinomas, including their epidemiology, clinical manifestations, diagnosis, staging, treatment, and prognosis. It provides details on: the average age of diagnosis being 60-70 years old; the most common histologic subtype being intestinal (47%); obstructive jaundice being the most common presenting symptom (80%); diagnostic tests including ERCP, CT, and tumor markers; the TNM staging system; pancreaticoduodenectomy being the standard treatment for localized disease; and adjuvant therapy options including chemotherapy and chemoradiotherapy for stage IB or higher cancers. […] The average age at diagnosis of sporadic ampullary carcinomas is 60 to 70 years old. […] In one study of 170 ampullary carcinomas, the most common histologic subtype was intestinal (47 percent), followed by pancreatobiliary (24 percent), poorly differentiated adenocarcinomas (13 percent), intestinal-mucinous (8 percent), and invasive papillary (5 percent).

• #17

  https://journals.lww.com/ajg/fulltext/2021/10001/s1373_epidemiology_of_ampullary_cancer_in_the.1377.aspx

  Ampullary cancer is rare and account for 0.2% of gastrointestinal tract tumors with estimated average 5-year survival rate of 40%. […] There are limited data in the literature regarding the epidemiology of ampullary cancer. […] Prevalence of Ampullary cancer is 4.01 per 100.000. […] After adjusting for cofounding factors, patients with ampullary cancer are more likely to be senior adults with age above 65 (OR 5.4, 95% CI: 4.8 – 6.1), males (OR 1.4, 95% CI: 1.3 – 1.6), smokers (OR 1.7, 95% CI: 1.5-1.9), diabetic (OR 2.6, 95% CI: 2.4 – 2.9), and have inflammatory bowel disease (OR 5.6, 95% CI: 4.7 – 7), with family history of gastrointestinal malignancy (OR 4.5, 95% CI: 3.8 – 5.2). […] Our study is one of the largest US population studies to date evaluating the epidemiology of ampullary cancer. […] Patients with ampullary cancer were more likely to be elderly, males, asian, smokers, diabetic, and have inflammatory bowel disease and with family history of gastrointestinal malignancy.

• #18

  https://journals.lww.com/ajg/fulltext/2021/10001/s1373_epidemiology_of_ampullary_cancer_in_the.1377.aspx

  Ampullary cancer is rare and account for 0.2% of gastrointestinal tract tumors with estimated average 5-year survival rate of 40%. […] There are limited data in the literature regarding the epidemiology of ampullary cancer. […] Prevalence of Ampullary cancer is 4.01 per 100.000. […] After adjusting for cofounding factors, patients with ampullary cancer are more likely to be senior adults with age above 65 (OR 5.4, 95% CI: 4.8 – 6.1), males (OR 1.4, 95% CI: 1.3 – 1.6), smokers (OR 1.7, 95% CI: 1.5-1.9), diabetic (OR 2.6, 95% CI: 2.4 – 2.9), and have inflammatory bowel disease (OR 5.6, 95% CI: 4.7 – 7), with family history of gastrointestinal malignancy (OR 4.5, 95% CI: 3.8 – 5.2). […] Our study is one of the largest US population studies to date evaluating the epidemiology of ampullary cancer. […] Patients with ampullary cancer were more likely to be elderly, males, asian, smokers, diabetic, and have inflammatory bowel disease and with family history of gastrointestinal malignancy.

• #19

  https://journals.lww.com/ajg/fulltext/2021/10001/s1373_epidemiology_of_ampullary_cancer_in_the.1377.aspx

  Ampullary cancer is rare and account for 0.2% of gastrointestinal tract tumors with estimated average 5-year survival rate of 40%. […] There are limited data in the literature regarding the epidemiology of ampullary cancer. […] Prevalence of Ampullary cancer is 4.01 per 100.000. […] After adjusting for cofounding factors, patients with ampullary cancer are more likely to be senior adults with age above 65 (OR 5.4, 95% CI: 4.8 – 6.1), males (OR 1.4, 95% CI: 1.3 – 1.6), smokers (OR 1.7, 95% CI: 1.5-1.9), diabetic (OR 2.6, 95% CI: 2.4 – 2.9), and have inflammatory bowel disease (OR 5.6, 95% CI: 4.7 – 7), with family history of gastrointestinal malignancy (OR 4.5, 95% CI: 3.8 – 5.2). […] Our study is one of the largest US population studies to date evaluating the epidemiology of ampullary cancer. […] Patients with ampullary cancer were more likely to be elderly, males, asian, smokers, diabetic, and have inflammatory bowel disease and with family history of gastrointestinal malignancy.

• #20 Epidemiology, clinical presentation, diagnosis, and staging of ampullary carcinoma – UpToDate

  https://www.uptodate.com/contents/ampullary-carcinoma-epidemiology-clinical-manifestations-diagnosis-and-staging

  Epidemiology, clinical features, diagnosis, and staging of ampullary carcinoma will be reviewed here. […] Neoplastic transformation of the intestinal mucosa occurs more commonly near the ampulla than at any other site in the small intestine. Despite this, primary ampullary tumors are rare, with an incidence of approximately 4 to 10 cases per million population. They account for only 6 percent of lesions that arise in the periampullary region but are responsible for 20 percent of all tumor-related obstructions of the common bile duct. There is some evidence that the incidence has increased over the last 30 years, although this has not been seen in all studies. […] Both benign and malignant ampullary tumors can occur sporadically or in the setting of a genetic syndrome. The incidence of ampullary tumors is increased 200- to 300-fold among patients with hereditary polyposis syndromes, such as familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome), compared with the general population. Surveillance endoscopy is particularly important to detect early ampullary lesions in patients with FAP given the high incidence of coexisting premalignant duodenal adenomatous polyps. Up to 90 percent of patients with FAP develop adenomas in the upper gastrointestinal tract.

• #21 Ampullary cancer – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ampullary-cancer/symptoms-causes/syc-20355066

  Ampullary cancer is cancer that starts as a growth of cells in the ampulla of Vater. Ampullary (AM-poo-la-ree) cancer is rare. […] Ampullary cancer forms near many other parts of the digestive system. This includes the liver, pancreas and small intestine. When ampullary cancer grows, it may affect these other organs. […] Factors that can increase the risk of ampullary cancer include: Increasing age. Ampullary cancer is more common in adults older than 70. […] DNA changes that run in families. Some DNA changes that are passed down from your parents can increase your risk of ampullary cancer and other cancers. Examples include the DNA changes that are linked to Lynch syndrome, familial adenomatous polyposis, also called FAP, and Peutz-Jeghers syndrome. […] There is no way to prevent ampullary cancer. […] Martin JA. Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging. https://www.uptodate.com/contents/search. Accessed Aug. 29, 2023.

• #22 Epidemiology, clinical presentation, diagnosis, and staging of ampullary carcinoma – UpToDate

  https://www.uptodate.com/contents/ampullary-carcinoma-epidemiology-clinical-manifestations-diagnosis-and-staging

  Epidemiology, clinical features, diagnosis, and staging of ampullary carcinoma will be reviewed here. […] Neoplastic transformation of the intestinal mucosa occurs more commonly near the ampulla than at any other site in the small intestine. Despite this, primary ampullary tumors are rare, with an incidence of approximately 4 to 10 cases per million population. They account for only 6 percent of lesions that arise in the periampullary region but are responsible for 20 percent of all tumor-related obstructions of the common bile duct. There is some evidence that the incidence has increased over the last 30 years, although this has not been seen in all studies. […] Both benign and malignant ampullary tumors can occur sporadically or in the setting of a genetic syndrome. The incidence of ampullary tumors is increased 200- to 300-fold among patients with hereditary polyposis syndromes, such as familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome), compared with the general population. Surveillance endoscopy is particularly important to detect early ampullary lesions in patients with FAP given the high incidence of coexisting premalignant duodenal adenomatous polyps. Up to 90 percent of patients with FAP develop adenomas in the upper gastrointestinal tract.

• #23 Molecular Pathology of Ampullary, Intra-Pancreatic Bile Duct and Duodenal Cancers | SpringerLink

  https://link.springer.com/10.1007%2F978-0-387-77498-5_9

  Periampullary cancers include carcinomas of the pancreatic head, ampullary cancers at the papilla of Vater, duodenal cancers and distal bile duct cancers. […] However, striking differences in survival are consistently being observed between the different periampullary tumor types, and histological classification is sometimes difficult due to overlapping histopathological phenotypes. […] In contrast, data on the molecular patterns characterizing sporadic periampullary non-pancreatic cancers are still fragmentary and further studies are required to better understand their genetic, transcriptional and epigenetic characteristics. […] Periampullary cancers may be associated with familial cancer syndromes such as familial adenomatous polyposis (FAP) or hereditary non-polypous colon cancer (HNPCC).

• #24

  https://journals.lww.com/ajg/fulltext/2018/10001/demographics,_tumor_characteristics,_treatment,.85.aspx

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers. […] The overall age-adjusted incidence of ampullary cancer between 2004 and 2014 was 0.59 per 100,000 per year. […] A constant incidence rate was noted during the study period; however, a higher incidence of ampullary tumors was observed in males compared to females (0.74 vs 0.48 per 100,000 per year). […] One and 5-year cause-specific survival for ampullary cancer was 71.7% and 38.8 respectively, with a median survival of 31 months. […] Ampullary cancer is rare and has a low survival rate. Its incidence increases with age, however, those treated surgically tend to have better outcomes.

• #25 Ampullary Cancer: An Overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4966534/

  Ampullary cancers are rare, accounting for only 0.2% of gastrointestinal cancers and approximately 7% of all periampullary cancers. They arise from the ampullary complex, distal to the confluence of the common bile and pancreatic duct. In contrast to other periampullary malignancies, true ampullary cancers present earlier in their disease course with symptoms that result from biliary obstruction. It is often difficult to distinguish primary ampullary cancers from other periampullary cancers preoperatively. In early stages, ampullary cancers are surgically treated, similar to pancreatic cancers, and typically with a pancreatico-duodenoectomy (or Whipple procedure). Because of their earlier presentation, resection rates for all patients are much higher than other periampullary carcinomas. Moreover, their prognosis tends to be better than those with other periampullary- and pancreatic-originating cancers. In patients with true ampullary cancer, there is very limited data to guide physicians on the choice of therapy, largely because of the rarity of the disease and the paucity of related research.

• #26 Ampullary cancer: 9 things to know about this rare gastrointestinal cancer | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/ampullary-cancer-9-insights-on-this-rare-gastrointestinal-cancer.h00-159542112.html

  Ampullary cancer is a rare type of gastrointestinal cancer with symptoms so similar to pancreatic cancer that it is sometimes mistaken for the disease. […] To learn more about this uncommon disease that accounts for only 1% of all gastrointestinal cancers, we spoke with Michael Overman, M.D., who treats patients in MD Andersons Gastrointestinal Cancer Center. […] The good news is that jaundice leads people to visit their doctors. And because jaundice is usually the first sign of ampullary cancer, the disease is often caught early. This results in a better prognosis for ampullary cancer, compared to pancreatic cancer and other types of gastrointestinal cancers. […] Its not clear what causes ampullary cancer. But we know that people with inherited conditions like familial adenomatous polyposis (FAP) or Lynch syndrome that cause polyps to grow in the digestive system are more likely to develop the disease.

• #27 Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 21 Issue 7 (2023)

  https://jnccn.org/abstract/journals/jnccn/21/7/article-p753.xml

  Ampullary cancers refer to tumors originating from the ampulla of Vater (the ampulla, the intraduodenal portion of the bile duct, and the intraduodenal portion of the pancreatic duct), while periampullary cancers may arise from locations encompassing the head of the pancreas, distal bile duct, duodenum, or ampulla of Vater. Ampullary cancers are rare gastrointestinal malignancies, and prognosis varies greatly based on factors such as patient age, TNM classification, differentiation grade, and treatment modality received. […] The 5-year overall survival (OS) for ampullary cancer is between 35% and 50%; however, prognosis can vary greatly based on a variety of factors such as patient age, TNM classification, differentiation grade, and treatment modality received. […] For example, the 5-year OS for AJCC 7th Edition stage I, stage II, and stage III + IV ampullary cancers is 64%, 27%, and 17%, respectively.

• #28 Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 21 Issue 7 (2023)

  https://jnccn.org/abstract/journals/jnccn/21/7/article-p753.xml

  Ampullary cancers refer to tumors originating from the ampulla of Vater (the ampulla, the intraduodenal portion of the bile duct, and the intraduodenal portion of the pancreatic duct), while periampullary cancers may arise from locations encompassing the head of the pancreas, distal bile duct, duodenum, or ampulla of Vater. Ampullary cancers are rare gastrointestinal malignancies, and prognosis varies greatly based on factors such as patient age, TNM classification, differentiation grade, and treatment modality received. […] The 5-year overall survival (OS) for ampullary cancer is between 35% and 50%; however, prognosis can vary greatly based on a variety of factors such as patient age, TNM classification, differentiation grade, and treatment modality received. […] For example, the 5-year OS for AJCC 7th Edition stage I, stage II, and stage III + IV ampullary cancers is 64%, 27%, and 17%, respectively.

• #29 The Diagnosis and Treatment of Ampullary Carcinoma (27.10.2023)

  https://di.aerzteblatt.de/int/archive/article/234965

  Ampullary carcinoma has an incidence of 0.5 to 0.9 per 100 000 persons and a poor prognosis, with a 5-year survival rate of 41% to 45% for locally confined and 4% to 7% for metastatic disease. […] The ICD-10 code for ampullary carcinomas is C24.1; the cancer data from the RKI have to date not reported a separate incidence. For extrahepatic cholangiocarcinomas (together with C24.0, C22.8, and C22.9) the incidence in Germany in 2015 was 2.9/100 000 in men and 1.8/100 000 in women. […] Five-year survival rates varied between 10% and 75% in the localized stage and 4.7% in the metastatic stage. […] The treatment of choice in ampullary carcinoma is radical oncological resection of the head of the pancreas with systematic lymphadenectomy. […] The poor prognosis even at the early resectable stage prompts the question of whether adjuvant therapeutic concepts can potentially improve the disease course.

• #30 Ampullary Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276413-overview

  Ampullary carcinoma is a relatively uncommon tumor that accounts for approximately 0.2% of gastrointestinal tract malignancies and approximately 7% of all periampullary carcinomas in the United States. A review of data from the National Cancer Institutes Surveillance, Epidemiology and End Results (SEER) program found 6803 patients with ampullary cancer between 2004 and 2013; the annual incidence has been fairly constant since 2004. […] An epidemiologic study of ampullary cancer in the Netherlands found that the incidence rate increased from 0.59 per 100,000 in 1989-1995 to 0.68 per 100,000 in 2010-2016. In patients with non-metastatic ampullary disease, five-year overall survival increased from 19.8% in 1989-1995 to 29.1% in 2010-2016. […] Because ampullary carcinoma is relatively uncommon, studies of the patterns of occurrence among different ethnic groups have not been conducted. Ampullary cancer is more common in men, according to the SEER program.

• #31 A population-based study on incidence, treatment, and survival in ampullary cancer in the Netherlands

  https://dspace.library.uu.nl/handle/1874/442430

  Ampullary cancer is rare and as a result epidemiological data are scarce. The aim of this population-based study was to determine the trends in incidence, treatment and overall survival (OS) in patients with ampullary adenocarcinoma in the Netherlands between 1989 and 2016. […] In total, 3840 patients with ampullary adenocarcinoma were diagnosed of whom, 55.0% were male and 87.1% had non-metastatic disease. The incidence increased from 0.59 per 100,000 in 19891995 to 0.68 per 100,000 in 20102016. […] Both incidence and OS of ampullary cancer increased from 1989 to 2016 which is most likely related to the observed increased resection rates and use of adjuvant therapy.

• #32 The pathohistological subtype strongly predicts survival in patients with ampullary carcinoma | Scientific Reports

  https://www.nature.com/articles/s41598-019-49179-w

  Ampullary cancer represents approximately 6% of the malignant periampullary tumors. An early occurrence of symptoms leads to a 5-year survival rate after curative surgery of 30 to 67%. In addition to the tumor stage, the immunohistological subtypes appear to be important for postoperative prognosis. The pathohistological subtypes of ampullary cancer allows a prediction of the postoperative prognosis. Ampullary cancer is a rare malignant disease, occurring in approximately 0.2% of all gastrointestinal tumors. Its localization characteristic symptoms like jaundice and pain usually occur earlier compared with other malignant pancreatobiliary tumors like pancreatic cancer. This is possibly the reason for the better prognosis of ampullary carcinomas with a reported 5-year survival rate of 45% in resected patients. According to the histopathologic characteristics, different subtypes of ampullary carcinomas are described. The pancreatobiliary subtype arises from simple mucinous epithelium of the distal common bile duct, the distal pancreatic duct, or common ampullary duct with simple or branching glands and small solid cell-nests enclosed by desmoplastic stroma. When comparing the subtypes, they differ in prognosis with 5-year survival rates ranging from 20% for the pancreatobiliary subtype to 88% for the intestinal subtype for resected patients. The pancreatobiliary subtype is significantly associated with a generally higher tumor stage at the time of presentation compared with the other subtypes. The survival analysis of the different subtypes was calculated using the Kaplan-Meier method with log rank test. The mean survival time for the pancreatobiliary subtype was 52 months, for the intestinal subtype 115 months, and for the mixed subtype 94 months. A significantly worse 5-year survival rate was found for the pancreatobiliary subtype with 27.5% compared with the intestinal subtype with 61%. The subtype was also found to be an independent survival-predicting risk factor. The poorer prognosis of the pancreatobiliary subtype could probably be explained by a disseminating growth behavior, perineural sheet invasion, and by a distinct desmoplastic stromal reaction. The pancreatobiliary subtype is associated with poor prognosis and a higher rate of lymph node metastasis, whereas the intestinal subtype shows an excellent prognosis. Prospective studies are recommended to evaluate the effect of subtype-stratified adjuvant chemotherapy.

• #33 The pathohistological subtype strongly predicts survival in patients with ampullary carcinoma | Scientific Reports

  https://www.nature.com/articles/s41598-019-49179-w

  Ampullary cancer represents approximately 6% of the malignant periampullary tumors. An early occurrence of symptoms leads to a 5-year survival rate after curative surgery of 30 to 67%. In addition to the tumor stage, the immunohistological subtypes appear to be important for postoperative prognosis. The pathohistological subtypes of ampullary cancer allows a prediction of the postoperative prognosis. Ampullary cancer is a rare malignant disease, occurring in approximately 0.2% of all gastrointestinal tumors. Its localization characteristic symptoms like jaundice and pain usually occur earlier compared with other malignant pancreatobiliary tumors like pancreatic cancer. This is possibly the reason for the better prognosis of ampullary carcinomas with a reported 5-year survival rate of 45% in resected patients. According to the histopathologic characteristics, different subtypes of ampullary carcinomas are described. The pancreatobiliary subtype arises from simple mucinous epithelium of the distal common bile duct, the distal pancreatic duct, or common ampullary duct with simple or branching glands and small solid cell-nests enclosed by desmoplastic stroma. When comparing the subtypes, they differ in prognosis with 5-year survival rates ranging from 20% for the pancreatobiliary subtype to 88% for the intestinal subtype for resected patients. The pancreatobiliary subtype is significantly associated with a generally higher tumor stage at the time of presentation compared with the other subtypes. The survival analysis of the different subtypes was calculated using the Kaplan-Meier method with log rank test. The mean survival time for the pancreatobiliary subtype was 52 months, for the intestinal subtype 115 months, and for the mixed subtype 94 months. A significantly worse 5-year survival rate was found for the pancreatobiliary subtype with 27.5% compared with the intestinal subtype with 61%. The subtype was also found to be an independent survival-predicting risk factor. The poorer prognosis of the pancreatobiliary subtype could probably be explained by a disseminating growth behavior, perineural sheet invasion, and by a distinct desmoplastic stromal reaction. The pancreatobiliary subtype is associated with poor prognosis and a higher rate of lymph node metastasis, whereas the intestinal subtype shows an excellent prognosis. Prospective studies are recommended to evaluate the effect of subtype-stratified adjuvant chemotherapy.

• #34 Survival and Prognostic Factors in Pancreatic and Ampullary Cancer | Anticancer Research

  https://ar.iiarjournals.org/content/34/6/3011

  Aim: We analyzed survival of patients diagnosed with ampullary cancer (AC) and pancreatic ductal adenocarcinomas (PDAC). […] The 5-year overall survival rate of patients with AC (37%; 95% confidence interval 25-49%) was remarkably higher compared to PDAC patients (7%; 95% confidence interval 5-10%). […] Interestingly, absence of lymph node metastasis substantially improved survival in AC, but not in PDAC. […] Malignant neoplasm of the ampulla of Vater, the shared outlet of the pancreatic and biliary duct, is a distinct tumor entity and referred to as ampullary carcinoma (AC). AC is in most cases an adenocarcinoma with heterogeneous origin arising either from the epithelial cells of the biliary or pancreatic duct (pancreatobiliary type), or from the duodenal mucosa (intestinal type). Its incidence is lower than PDAC, but prognosis of patients diagnosed with AC is considered to be superior to that for PDAC.

• #35 Survival and Prognostic Factors in Pancreatic and Ampullary Cancer | Anticancer Research

  https://ar.iiarjournals.org/content/34/6/3011

  Aim: We analyzed survival of patients diagnosed with ampullary cancer (AC) and pancreatic ductal adenocarcinomas (PDAC). […] The 5-year overall survival rate of patients with AC (37%; 95% confidence interval 25-49%) was remarkably higher compared to PDAC patients (7%; 95% confidence interval 5-10%). […] Interestingly, absence of lymph node metastasis substantially improved survival in AC, but not in PDAC. […] Malignant neoplasm of the ampulla of Vater, the shared outlet of the pancreatic and biliary duct, is a distinct tumor entity and referred to as ampullary carcinoma (AC). AC is in most cases an adenocarcinoma with heterogeneous origin arising either from the epithelial cells of the biliary or pancreatic duct (pancreatobiliary type), or from the duodenal mucosa (intestinal type). Its incidence is lower than PDAC, but prognosis of patients diagnosed with AC is considered to be superior to that for PDAC.

• #36 Survival and Prognostic Factors in Pancreatic and Ampullary Cancer | Anticancer Research

  https://ar.iiarjournals.org/content/34/6/3011

  Aim: We analyzed survival of patients diagnosed with ampullary cancer (AC) and pancreatic ductal adenocarcinomas (PDAC). […] The 5-year overall survival rate of patients with AC (37%; 95% confidence interval 25-49%) was remarkably higher compared to PDAC patients (7%; 95% confidence interval 5-10%). […] Interestingly, absence of lymph node metastasis substantially improved survival in AC, but not in PDAC. […] Malignant neoplasm of the ampulla of Vater, the shared outlet of the pancreatic and biliary duct, is a distinct tumor entity and referred to as ampullary carcinoma (AC). AC is in most cases an adenocarcinoma with heterogeneous origin arising either from the epithelial cells of the biliary or pancreatic duct (pancreatobiliary type), or from the duodenal mucosa (intestinal type). Its incidence is lower than PDAC, but prognosis of patients diagnosed with AC is considered to be superior to that for PDAC.

• #37 Epidemiology, clinical presentation, diagnosis, and staging of ampullary carcinoma – UpToDate

  https://www.uptodate.com/contents/ampullary-carcinoma-epidemiology-clinical-manifestations-diagnosis-and-staging

  Epidemiology, clinical features, diagnosis, and staging of ampullary carcinoma will be reviewed here. […] Neoplastic transformation of the intestinal mucosa occurs more commonly near the ampulla than at any other site in the small intestine. Despite this, primary ampullary tumors are rare, with an incidence of approximately 4 to 10 cases per million population. They account for only 6 percent of lesions that arise in the periampullary region but are responsible for 20 percent of all tumor-related obstructions of the common bile duct. There is some evidence that the incidence has increased over the last 30 years, although this has not been seen in all studies. […] Both benign and malignant ampullary tumors can occur sporadically or in the setting of a genetic syndrome. The incidence of ampullary tumors is increased 200- to 300-fold among patients with hereditary polyposis syndromes, such as familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome), compared with the general population. Surveillance endoscopy is particularly important to detect early ampullary lesions in patients with FAP given the high incidence of coexisting premalignant duodenal adenomatous polyps. Up to 90 percent of patients with FAP develop adenomas in the upper gastrointestinal tract.

• #38 Surveillance Rather Than Immediate Resection Appears Advisable for Most Familial Adenomatous Polyposis-Related Ampullary Adenomas – Endoscopy Campus

  https://www.endoscopy-campus.com/en/ec-news/surveillance-rather-than-immediate-resection-appears-advisable-for-most-familial-adenomatous-polyposis-related-ampullary-adenomas/

  Ampullary, periampullary, and duodenal adenomas commonly occur in familial adenomatous polyposis (FAP). Their detection and management are fraught with challenges related to balancing the risk of progression to carcinoma and risks of endoscopic management. […] Among 143 patients followed for a median of 7.8 years, SP occurred in 28.6%, including growth in size to 10 mm in 15.6%, development of advanced histology in 8.5%, and changes in both histology and size in 4.9%. Ampullary cancer developed in 2 of 143 patients (1.4%). […] Based on these data, the authors advise ongoing endoscopic surveillance, rather than early resection, for most ampullary adenomas in those with FAP.

• #39 Surveillance Rather Than Immediate Resection Appears Advisable for Most Familial Adenomatous Polyposis-Related Ampullary Adenomas – Endoscopy Campus

  https://www.endoscopy-campus.com/en/ec-news/surveillance-rather-than-immediate-resection-appears-advisable-for-most-familial-adenomatous-polyposis-related-ampullary-adenomas/

  Ampullary, periampullary, and duodenal adenomas commonly occur in familial adenomatous polyposis (FAP). Their detection and management are fraught with challenges related to balancing the risk of progression to carcinoma and risks of endoscopic management. […] Among 143 patients followed for a median of 7.8 years, SP occurred in 28.6%, including growth in size to 10 mm in 15.6%, development of advanced histology in 8.5%, and changes in both histology and size in 4.9%. Ampullary cancer developed in 2 of 143 patients (1.4%). […] Based on these data, the authors advise ongoing endoscopic surveillance, rather than early resection, for most ampullary adenomas in those with FAP.

• #40 Surveillance Rather Than Immediate Resection Appears Advisable for Most Familial Adenomatous Polyposis-Related Ampullary Adenomas – Endoscopy Campus

  https://www.endoscopy-campus.com/en/ec-news/surveillance-rather-than-immediate-resection-appears-advisable-for-most-familial-adenomatous-polyposis-related-ampullary-adenomas/

  Ampullary, periampullary, and duodenal adenomas commonly occur in familial adenomatous polyposis (FAP). Their detection and management are fraught with challenges related to balancing the risk of progression to carcinoma and risks of endoscopic management. […] Among 143 patients followed for a median of 7.8 years, SP occurred in 28.6%, including growth in size to 10 mm in 15.6%, development of advanced histology in 8.5%, and changes in both histology and size in 4.9%. Ampullary cancer developed in 2 of 143 patients (1.4%). […] Based on these data, the authors advise ongoing endoscopic surveillance, rather than early resection, for most ampullary adenomas in those with FAP.

• #41 Ampullary cancer – Pathway

  https://m.pathway.md/diseases/ampullary-cancer-rec3CEGnDJZsVsVtK

  The incidence of ampullary cancer in the Unites States is estimated at 0.59 per 100,000 person-years. […] Postoperative surveillance: as per ESGE 2021 guidelines, obtain long-term monitoring of patients after endoscopic papillectomy or surgical ampullectomy, based on duodenoscopy with biopsies of the scar and of any abnormal area, within the first 3 months, at 6 and 12 months, and thereafter yearly for at least 5 years.

• #42 PRIMARY AMPULLARY CANCER: A RARE CAUSE OF ABDOMINAL PAIN – SHM Abstracts | Society of Hospital Medicine

  https://shmabstracts.org/abstract/primary-ampullary-cancer-a-rare-cause-of-abdominal-pain/

  Primary ampullary tumor has low incidence of approximately four per million. […] Currently, there is no defined optimal surveillance. Most clinicians utilize CA19-9 or CEA and repeat CT scan every six months for the first five years. […] It is important to identify primary ampullary cancer with ERCP and biopsy due to favorable prognosis. In addition to surgical resection, chemoradiotherapy or chemotherapy are widely accepted management. These patients should be followed with repeat tumor markers and image studies.

• #43 Ampullary Cancer: Risk Factors, Treatments, and Prognosis

  https://www.healthline.com/health/cancer/ampullary-cancer

  An estimated 45 percent of people treated with surgery for ampullary cancer experience a recurrence of their cancer. If a person has a tumor at stage T2 or higher, a doctor will usually recommend chemotherapy to prevent cancer recurrence. […] Ampullary cancer is a rare gastrointestinal cancer that has a good outlook with early treatment but is known to recur.

• #44 The pathohistological subtype strongly predicts survival in patients with ampullary carcinoma | Scientific Reports

  https://www.nature.com/articles/s41598-019-49179-w

  Ampullary cancer represents approximately 6% of the malignant periampullary tumors. An early occurrence of symptoms leads to a 5-year survival rate after curative surgery of 30 to 67%. In addition to the tumor stage, the immunohistological subtypes appear to be important for postoperative prognosis. The pathohistological subtypes of ampullary cancer allows a prediction of the postoperative prognosis. Ampullary cancer is a rare malignant disease, occurring in approximately 0.2% of all gastrointestinal tumors. Its localization characteristic symptoms like jaundice and pain usually occur earlier compared with other malignant pancreatobiliary tumors like pancreatic cancer. This is possibly the reason for the better prognosis of ampullary carcinomas with a reported 5-year survival rate of 45% in resected patients. According to the histopathologic characteristics, different subtypes of ampullary carcinomas are described. The pancreatobiliary subtype arises from simple mucinous epithelium of the distal common bile duct, the distal pancreatic duct, or common ampullary duct with simple or branching glands and small solid cell-nests enclosed by desmoplastic stroma. When comparing the subtypes, they differ in prognosis with 5-year survival rates ranging from 20% for the pancreatobiliary subtype to 88% for the intestinal subtype for resected patients. The pancreatobiliary subtype is significantly associated with a generally higher tumor stage at the time of presentation compared with the other subtypes. The survival analysis of the different subtypes was calculated using the Kaplan-Meier method with log rank test. The mean survival time for the pancreatobiliary subtype was 52 months, for the intestinal subtype 115 months, and for the mixed subtype 94 months. A significantly worse 5-year survival rate was found for the pancreatobiliary subtype with 27.5% compared with the intestinal subtype with 61%. The subtype was also found to be an independent survival-predicting risk factor. The poorer prognosis of the pancreatobiliary subtype could probably be explained by a disseminating growth behavior, perineural sheet invasion, and by a distinct desmoplastic stromal reaction. The pancreatobiliary subtype is associated with poor prognosis and a higher rate of lymph node metastasis, whereas the intestinal subtype shows an excellent prognosis. Prospective studies are recommended to evaluate the effect of subtype-stratified adjuvant chemotherapy.

• #45 Ampullary Cancer: Histological Subtypes, Markers, and Clinical Behaviour—State of the Art and Perspectives

  https://www.mdpi.com/1718-7729/30/7/507

  Ampullary adenocarcinoma (AAC) is a rare disease, accounting for only 0.2% of gastrointestinal malignancies and 6% of periampullary tumours. […] The objective of this article is to critically review the available literature concerning the two histological subtypes of AAC, particularly focusing on (a) their histological and immunohistochemical features; (b) their impacts on prognoses; (c) the roles of adjuvant treatments for each subtype. […] Currently, the last classifications of ampullary cancer were distinguished separately into the intestinal (int) and pancreato-biliary (pb) subtypes. […] It is important to underscore that this distinction of AAC is not accepted by some authors: an analysis of the surveillance, epidemiology, and end results program of the national cancer institute of USA (SEER) database by Albores-Saavedra et al. in 2009 demonstrated that AAC shows the same carcinogenic epidemiological pattern, independent of its histological diagnosis, suggesting a single population of AAC and similar or overlapping carcinogenic pathways.

• #46 Patterns of failure for stage I ampulla of Vater adenocarcinoma: a single institutional experience – Zhong – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/3314/3825

  Ampullary adenocarcinoma is a rare malignancy associated with a relatively favorable prognosis. Given high survival rates in stage I patients reported in small series with surgery alone, adjuvant chemoradiotherapy (CRT) has traditionally been recommended only for patients with high risk disease. Recent population-based data have demonstrated inferior outcomes to previous series. We examined disease-related outcomes for stage I tumors treated with pancreaticoduodenectomy, with and without CRT. […] The prognosis for patients diagnosed with stage I ampullary adenocarcinoma may not be as favorable as previously described. Our data suggests a possible benefit of adjuvant CRT delivery. […] Given that 39% of patients who received surgery alone experienced locoregional failure, we believe there is a clinically significant risk of residual subclinical disease following radical resection, even for early tumors. These findings are consistent with a previous study suggesting that local resection for early ampullary tumors is inadequate in preventing disease recurrence. In our series, the addition of adjuvant CRT appeared to reduce local failure rates, although not statistically significant, given small patient numbers. Additionally, there appeared to be a large number of patients who developed distant metastases, most commonly in the liver.

• #47 Patterns of failure for stage I ampulla of Vater adenocarcinoma: a single institutional experience – Zhong – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/3314/3825

  Ampullary adenocarcinoma is a rare malignancy associated with a relatively favorable prognosis. Given high survival rates in stage I patients reported in small series with surgery alone, adjuvant chemoradiotherapy (CRT) has traditionally been recommended only for patients with high risk disease. Recent population-based data have demonstrated inferior outcomes to previous series. We examined disease-related outcomes for stage I tumors treated with pancreaticoduodenectomy, with and without CRT. […] The prognosis for patients diagnosed with stage I ampullary adenocarcinoma may not be as favorable as previously described. Our data suggests a possible benefit of adjuvant CRT delivery. […] Given that 39% of patients who received surgery alone experienced locoregional failure, we believe there is a clinically significant risk of residual subclinical disease following radical resection, even for early tumors. These findings are consistent with a previous study suggesting that local resection for early ampullary tumors is inadequate in preventing disease recurrence. In our series, the addition of adjuvant CRT appeared to reduce local failure rates, although not statistically significant, given small patient numbers. Additionally, there appeared to be a large number of patients who developed distant metastases, most commonly in the liver.

• #48 Ampullary Cancer: An Overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4966534/

  Curative surgery is possible in approximately 50% of ampullary cancer compared to that of less than 10% in pancreatic adenocarcinoma. Despite the high rate of potentially curative resection, the majority of patients with ampullary carcinomas will eventually succumb to recurrent disease. Given the rarity of this disease, there is absence of randomized clinical trials focused on ampullary carcinomas and treatment recommendations are mainly derived from results of adjuvant clinical trials conducted in pancreaticobiliary cancers where ampullary cancers may represent a sub-group of patients. […] Systemic chemotherapy remains the mainstay in the treatment of patients with locally advanced unresectable and metastatic disease. Given the rarity of this disease, our reliance is on limited published data sets including a variety of trials including basket trials that allowed the inclusion of ampullary cancer in addition to small intestinal or biliary duct cancers. Agents that have been examined in this disease include anti-metabolites (fluoropyrimidine and/or gemcitabine) with or without the addition of a platinum compound (usually cisplatin or oxaliplatin). Published work suggests variability in response rates ranging from 10% to 40% and reported median overall survival up to 20 months.

• #49 Ampullary Adenocarcinoma: A Review of the Mutational Landscape and Implications for Treatment

  https://www.mdpi.com/2072-6694/15/24/5772

  Ampullary carcinomas represent less than 1% of all gastrointestinal malignancies with an incidence of approximately 6 cases per 1 million. […] According to the surveillance, epidemiology and end results (SEER) registry, ampullary adenocarcinoma has an annual incidence rate of approximately 6 cases per 1 million. […] Over 80% of ampullary carcinomas are resectable, with 5-year survival rates reaching over 30–50%, which is significantly higher than the rates of resection and survival observed in cholangiocarcinoma and pancreatic cancer. […] However, despite the higher resectability rates of ampullary adenocarcinomas, the majority of patients with resected ampullary carcinoma will still succumb to their disease. […] Multi-institutional collaboration will be necessary to better understand how molecular classification can guide type and sequencing of multimodality therapy.

• #50 Updates in Management of Ampullary Carcinomas

  https://www.primescholars.com/articles/updates-in-management-of-ampullary-carcinomas-97508.html

  Ampullary carcinomas are rare malignancies representing less than 1% of all gastrointestinal cancers. […] However, the incidence rate of ampullary cancers has increased by about 1% every year over the past 30 years. […] The standard treatment for ampullary carcinoma has been complete surgical resection via pancreaticoduodenectomy. […] Evidence suggests that pancreaticoduodenectomy has significantly improved survival rates in ampullary carcinomas with 5-year survival rates up to 39%. […] A recent prospective trial in 2010 analyzed survival of 450 patients with ampullary tumors who underwent pancreaticoduodenectomy and found that the 5-year survival rate was 45%. […] There has been a lack of specific therapeutic protocols for adjuvant treatment of ampullary cancer. […] A retrospective study from Narang et al. compared surgery plus adjuvant chemoradiation with surgery alone for patients with ampullary cancer.

• #51 Ampullary carcinoma | PPT

  https://www.slideshare.net/slideshow/ampullary-carcinoma/38417037

  K-ras mutations are an early event in ampullary carcinogenesis, with an incidence (37 percent) that is similar to that in colon cancer (up to 50 percent). […] The most common presenting symptom of ampullary carcinoma is obstructive jaundice (80 percent). […] The most commonly used staging system is the tumor-node-metastasis (TNM) system of the combined AJCC (American Joint Committee on Cancer)/UICC (International Union Against Cancer). […] The outcome of resected ampullary cancer depends upon 1. the extent of local invasion, 2. status of the surgical margins, 3. the presence or absence of nodal metastases. […] Resectability rates are higher, five-year survival rates are 30 to 50 percent in selected patients with limited lymph node involvement. […] The only potentially curative treatment for ampullary carcinoma is surgical resection.

• #52 Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 21 Issue 7 (2023)

  https://jnccn.org/abstract/journals/jnccn/21/7/article-p753.xml

  Regardless, ampullary tumors generally have a more favorable outcome compared with other periampullary malignancies. […] Early detection might partially contribute to this prognostic pattern. […] Systemic therapy is used in all stages of ampullary cancer. This includes neoadjuvant therapy for resectable or borderline resectable disease (albeit used more rarely compared with pancreatic cancer), adjuvant therapy, and first-line or subsequent-line therapy for locally advanced, metastatic, and recurrent disease. […] Data for systemic therapy in ampullary cancer are very limited; the only phase III randomized trial to date that enrolled a relatively large number of patients with ampullary cancer was ESPAC-3, which tested 5-fluorouracil (5-FU) + leucovorin versus gemcitabine in the adjuvant setting.

• #53 The future of adjuvant therapy in ampullary cancer: should we offer it to our patients? – Tella – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/31979/html

  Ampullary carcinoma is a relatively rare and heterogenous neoplasm accounting for 0.2% of all gastrointestinal tract malignancies. The data from National Cancer Institutes Surveillance, Epidemiology, and End Results Program have shown an increase in incidence of ampullary cancer over the last decade (1). […] However, the role of adjuvant therapy in ampullary carcinoma is not well established as the data is obtained either from therapeutic protocols employed to evaluate other peri-ampullary cancers or retrospective studies (5). […] Despite the controversy of the role of adjuvant therapy, there has been trend towards higher utilization of adjuvant therapy by clinicians in the United States, an increase from 9% to 32% in the years 2004-05 and 2012-13, respectively (6). […] The authors concluded that post-surgical adjuvant therapy has no role in improving OS in patients with ampullary cancer, irrespective of the stage at diagnosis, grade, resection margin status, lymph node positivity, and histologic subtype thereby questioning the adjuvant therapy use in clinical practice.

• #54 The future of adjuvant therapy in ampullary cancer: should we offer it to our patients? – Tella – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/31979/html

  Ampullary carcinoma is a relatively rare and heterogenous neoplasm accounting for 0.2% of all gastrointestinal tract malignancies. The data from National Cancer Institutes Surveillance, Epidemiology, and End Results Program have shown an increase in incidence of ampullary cancer over the last decade (1). […] However, the role of adjuvant therapy in ampullary carcinoma is not well established as the data is obtained either from therapeutic protocols employed to evaluate other peri-ampullary cancers or retrospective studies (5). […] Despite the controversy of the role of adjuvant therapy, there has been trend towards higher utilization of adjuvant therapy by clinicians in the United States, an increase from 9% to 32% in the years 2004-05 and 2012-13, respectively (6). […] The authors concluded that post-surgical adjuvant therapy has no role in improving OS in patients with ampullary cancer, irrespective of the stage at diagnosis, grade, resection margin status, lymph node positivity, and histologic subtype thereby questioning the adjuvant therapy use in clinical practice.

• #55 Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 21 Issue 7 (2023)

  https://jnccn.org/abstract/journals/jnccn/21/7/article-p753.xml

  Regardless, ampullary tumors generally have a more favorable outcome compared with other periampullary malignancies. […] Early detection might partially contribute to this prognostic pattern. […] Systemic therapy is used in all stages of ampullary cancer. This includes neoadjuvant therapy for resectable or borderline resectable disease (albeit used more rarely compared with pancreatic cancer), adjuvant therapy, and first-line or subsequent-line therapy for locally advanced, metastatic, and recurrent disease. […] Data for systemic therapy in ampullary cancer are very limited; the only phase III randomized trial to date that enrolled a relatively large number of patients with ampullary cancer was ESPAC-3, which tested 5-fluorouracil (5-FU) + leucovorin versus gemcitabine in the adjuvant setting.

• #56 The future of adjuvant therapy in ampullary cancer: should we offer it to our patients? – Tella – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/31979/html

  In the subgroup analysis of ampullary cancer patients in ESPAC-3 trial, OS benefit was observed only in gemcitabine arm and not in 5-fluorouracil arm (8). […] The controversy about the benefits of adjuvant therapy in ampullary cancer should be discussed with the patient and their goals of care are to be taken into consideration to deliver highest quality care to our patients.

• #57 Evaluation of adjuvant chemoradiation therapy for ampullary adenocarcinoma: the Johns Hopkins Hospital – Mayo Clinic collaborative study | Radiation Oncology | Full Text

  https://ro-journal.biomedcentral.com/articles/10.1186/1748-717X-6-126

  The role of adjuvant chemoradiation therapy for ampullary carcinoma is unknown. Previous literature suggests that certain populations with high risk factors for recurrence may benefit from adjuvant chemoradiation. […] Node-positive patients with resected ampullary adenocarcinoma may benefit from 5-FU based adjuvant chemoradiation. Since a significant proportion of patients develop metastatic disease, there is a need for more effective systemic treatment. […] Although carcinoma of the ampulla of Vater is a rare malignancy with an overall incidence of 6 in 1 million, it is the second most common periampullary cancer, comprising 6-20% of malignancies in this region. […] High rates of relapse along with identification of adverse prognostic factors have led to exploration of adjuvant chemoradiation for „high risk” ampullary carcinoma, although the literature in this area remains sparse.

• #58 Evaluation of adjuvant chemoradiation therapy for ampullary adenocarcinoma: the Johns Hopkins Hospital – Mayo Clinic collaborative study | Radiation Oncology | Full Text

  https://ro-journal.biomedcentral.com/articles/10.1186/1748-717X-6-126

  The addition of adjuvant chemoradiation likely improves survival in patients with high risk disease, particularly in those with lymph node involvement. Whether all patients with resectable ampullary carcinoma should be treated with adjuvant chemoradiation is subject to debate. Certainly, better systemic therapy is necessary to improve the high rate of distant metastasis found in this population.

• #59 Updates in Management of Ampullary Carcinomas

  https://www.primescholars.com/articles/updates-in-management-of-ampullary-carcinomas-97508.html

  The results of the study showed that adjuvant radiotherapy does not significantly improve median survival times if data is not adjusted for tumor stage. […] In contrast, the subgroup of patients who had T2 tumors and received radiotherapy had significantly improved median survival times. […] Despite the above mentioned limitations, the presented study provides vital information to the literature concerning adjuvant treatment of patients with ampullary cancer.

• #60 Ampullary Cancer: An Overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4966534/

  Curative surgery is possible in approximately 50% of ampullary cancer compared to that of less than 10% in pancreatic adenocarcinoma. Despite the high rate of potentially curative resection, the majority of patients with ampullary carcinomas will eventually succumb to recurrent disease. Given the rarity of this disease, there is absence of randomized clinical trials focused on ampullary carcinomas and treatment recommendations are mainly derived from results of adjuvant clinical trials conducted in pancreaticobiliary cancers where ampullary cancers may represent a sub-group of patients. […] Systemic chemotherapy remains the mainstay in the treatment of patients with locally advanced unresectable and metastatic disease. Given the rarity of this disease, our reliance is on limited published data sets including a variety of trials including basket trials that allowed the inclusion of ampullary cancer in addition to small intestinal or biliary duct cancers. Agents that have been examined in this disease include anti-metabolites (fluoropyrimidine and/or gemcitabine) with or without the addition of a platinum compound (usually cisplatin or oxaliplatin). Published work suggests variability in response rates ranging from 10% to 40% and reported median overall survival up to 20 months.

• #61 Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 21 Issue 7 (2023)

  https://jnccn.org/abstract/journals/jnccn/21/7/article-p753.xml

  Thus, the NCCN recommendations for systemic therapy options in ampullary cancer are frequently extrapolated from data in the setting of pancreatic cancer, colorectal cancer, and biliary tract cancer, as well as panel members clinical experience. […] Radiation therapy (RT) is another treatment modality that can be used in localized ampullary cancer, sometimes in combination with chemotherapy, but no high level evidence exists to support its utility. […] The goal of RT is to sterilize vessel margins, enhance the likelihood of a margin-negative resection, and/or provide adequate local control to prevent or delay progression or prevent local disease recurrence while minimizing the risk of RT exposure to surrounding organs at risk. […] Finally, palliation and supportive care are warranted to prevent and ameliorate suffering while ensuring optimal quality of life for patients with end-stage disease who have run out of options.

• #62 Ampullary Cancer: An Overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4966534/

  Curative surgery is possible in approximately 50% of ampullary cancer compared to that of less than 10% in pancreatic adenocarcinoma. Despite the high rate of potentially curative resection, the majority of patients with ampullary carcinomas will eventually succumb to recurrent disease. Given the rarity of this disease, there is absence of randomized clinical trials focused on ampullary carcinomas and treatment recommendations are mainly derived from results of adjuvant clinical trials conducted in pancreaticobiliary cancers where ampullary cancers may represent a sub-group of patients. […] Systemic chemotherapy remains the mainstay in the treatment of patients with locally advanced unresectable and metastatic disease. Given the rarity of this disease, our reliance is on limited published data sets including a variety of trials including basket trials that allowed the inclusion of ampullary cancer in addition to small intestinal or biliary duct cancers. Agents that have been examined in this disease include anti-metabolites (fluoropyrimidine and/or gemcitabine) with or without the addition of a platinum compound (usually cisplatin or oxaliplatin). Published work suggests variability in response rates ranging from 10% to 40% and reported median overall survival up to 20 months.

• #63 Progression of Metastatic, PD-L1–Positive, Ampullary Carcinoma with a Treatment Holiday from Off-Label Use of Pembrolizumab Therapy

  https://jhoponline.com/issue-archive/2020-issues/august-2020-vol-10-no-4/progression-of-metastatic-pd-l1-positive-ampullary-carcinoma-with-a-treatment-holiday-from-off-label-use-of-pembrolizumab-therapy

  Ampullary carcinoma, a rare type of tumor that arises from the ampulla of Vater, represents only 0.2% of all gastrointestinal (GI) tract neoplasms to date. […] Given the low incidence of ampullary carcinoma, a standard approach to treatment is not well-established and no clinical guidelines are available. […] The use of pembrolizumab in the setting of ampullary carcinoma has not been explicitly studied. […] Pembrolizumab has not been approved by the FDA for use in the setting of microsatellite-stable cholangiocarcinoma or ampullary carcinoma, but several studies have provided examples of PD-L1 expression in the setting of ampullary carcinoma. […] The findings from this case report may provide preliminary insights on the potential role of immunotherapy for the third-line treatment of metastatic ampullary carcinoma. Further studies are warranted to confirm these findings.

• #64 Progression of Metastatic, PD-L1–Positive, Ampullary Carcinoma with a Treatment Holiday from Off-Label Use of Pembrolizumab Therapy

  https://www.jhoponline.com/issue-archive/2020-issues/august-2020-vol-10-no-4/18303-progression-of-metastatic-pd-l1-positive-ampullary-carcinoma-with-a-treatment-holiday-from-off-label-use-of-pembrolizumab-therapy

  Ampullary carcinoma, a rare type of tumor that arises from the ampulla of Vater, represents only 0.2% of all gastrointestinal (GI) tract neoplasms to date. […] Given the low incidence of ampullary carcinoma, a standard approach to treatment is not well-established and no clinical guidelines are available. […] The use of pembrolizumab in the setting of ampullary carcinoma has not been explicitly studied. […] Pembrolizumab has not been approved by the FDA for use in the setting of microsatellite-stable cholangiocarcinoma or ampullary carcinoma, but several studies have provided examples of PD-L1 expression in the setting of ampullary carcinoma. […] Given the absence of studies investigating the use of immunotherapy in the setting of metastatic ampullary carcinoma, the optimal timeline for immunotherapy use is not yet defined. […] The findings from this case report may provide preliminary insights on the potential role of immunotherapy for the third-line treatment of metastatic ampullary carcinoma. Further studies are warranted to confirm these findings.

• #65 Molecular Targets and Therapies for Ampullary Cancer in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 2D (2024)

  https://jnccn.org/abstract/journals/jnccn/22/2D/article-e237051.xml

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers, but incidence has been increasing since 1970 at a rate of 0.5% to 0.9% per year. It remains uncertain how improved imaging and procedural modalities have contributed to this increase. Multiple meta-analyses of ampullary, hepatic, pancreatic, and biliary cancers demonstrate that most patients are male and aged 60 years, with an increasing incidence over the previous 3 decades. Black race, higher stage and grade, and nonsurgical treatment are associated with worsened clinical outcomes. […] Ampullary carcinomas are rare but increasing in incidence. Ampullary cancers have molecular alterations that guide choice of therapy, particularly in nonresectable cases. These alterations can be more common by subtype (intestinal, pancreaticobiliary, or mixed), and next-generation sequencing is recommended for all patients who cannot undergo surgery.

• #66 Ampullary carcinoma | PPT

  https://www.slideshare.net/slideshow/ampullary-carcinoma/38417037

  K-ras mutations are an early event in ampullary carcinogenesis, with an incidence (37 percent) that is similar to that in colon cancer (up to 50 percent). […] The most common presenting symptom of ampullary carcinoma is obstructive jaundice (80 percent). […] The most commonly used staging system is the tumor-node-metastasis (TNM) system of the combined AJCC (American Joint Committee on Cancer)/UICC (International Union Against Cancer). […] The outcome of resected ampullary cancer depends upon 1. the extent of local invasion, 2. status of the surgical margins, 3. the presence or absence of nodal metastases. […] Resectability rates are higher, five-year survival rates are 30 to 50 percent in selected patients with limited lymph node involvement. […] The only potentially curative treatment for ampullary carcinoma is surgical resection.

• #67 Molecular Targets and Therapies for Ampullary Cancer in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 2D (2024)

  https://jnccn.org/abstract/journals/jnccn/22/2D/article-e237051.xml

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers, but incidence has been increasing since 1970 at a rate of 0.5% to 0.9% per year. It remains uncertain how improved imaging and procedural modalities have contributed to this increase. Multiple meta-analyses of ampullary, hepatic, pancreatic, and biliary cancers demonstrate that most patients are male and aged 60 years, with an increasing incidence over the previous 3 decades. Black race, higher stage and grade, and nonsurgical treatment are associated with worsened clinical outcomes. […] Ampullary carcinomas are rare but increasing in incidence. Ampullary cancers have molecular alterations that guide choice of therapy, particularly in nonresectable cases. These alterations can be more common by subtype (intestinal, pancreaticobiliary, or mixed), and next-generation sequencing is recommended for all patients who cannot undergo surgery.

• #68 Molecular Targets and Therapies for Ampullary Cancer in: Journal of the National Comprehensive Cancer Network Volume 22 Issue 2D (2024)

  https://jnccn.org/abstract/journals/jnccn/22/2D/article-e237051.xml

  Ampullary cancer accounts for only 0.2% of gastrointestinal cancers, but incidence has been increasing since 1970 at a rate of 0.5% to 0.9% per year. It remains uncertain how improved imaging and procedural modalities have contributed to this increase. Multiple meta-analyses of ampullary, hepatic, pancreatic, and biliary cancers demonstrate that most patients are male and aged 60 years, with an increasing incidence over the previous 3 decades. Black race, higher stage and grade, and nonsurgical treatment are associated with worsened clinical outcomes. […] Ampullary carcinomas are rare but increasing in incidence. Ampullary cancers have molecular alterations that guide choice of therapy, particularly in nonresectable cases. These alterations can be more common by subtype (intestinal, pancreaticobiliary, or mixed), and next-generation sequencing is recommended for all patients who cannot undergo surgery.

• #69 Ampullary Adenocarcinoma: A Review of the Mutational Landscape and Implications for Treatment

  https://www.mdpi.com/2072-6694/15/24/5772

  Ampullary carcinomas represent less than 1% of all gastrointestinal malignancies with an incidence of approximately 6 cases per 1 million. […] According to the surveillance, epidemiology and end results (SEER) registry, ampullary adenocarcinoma has an annual incidence rate of approximately 6 cases per 1 million. […] Over 80% of ampullary carcinomas are resectable, with 5-year survival rates reaching over 30–50%, which is significantly higher than the rates of resection and survival observed in cholangiocarcinoma and pancreatic cancer. […] However, despite the higher resectability rates of ampullary adenocarcinomas, the majority of patients with resected ampullary carcinoma will still succumb to their disease. […] Multi-institutional collaboration will be necessary to better understand how molecular classification can guide type and sequencing of multimodality therapy.

• #70 Prognostic importance of numbers of retrieved lymph nodes and positive lymph nodes for ampulla of vater cancer (AVC) in 2347 patients from the Surveillance, Epidemiology, and End Results (SEER) database | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0244987

  The numbers of retrieved lymph nodes (RLNs) and positive lymph nodes (PLNs) had a significant impact on the survival of patients with ampulla of vater cancer (AVC), but the optimal numbers of the both were controversial. […] The study found that the optimal cut-off values of RLNs were 18 in the N0 cohort and 16 in N1 or entire cohort due to the highest 5-year overall survival (OS) rate and disease-specific survival (DSS) rate and the separation of survival curves (all P 0.05). […] This article recommended that at least 16 lymph nodes will improve the prognosis of AVC patients undergoing surgery. The best cut-off values of PLNs recommended for this study were 0 and 2, which may accurately stratify patients. […] The optimal cut-off value of RLNs supported by this study was 16. Adequate lymph node dissection may improve the prognosis of patients undergoing radical surgery for malignant tumors. Thus, routine lymph node dissection was recommended in AVC patients who underwent surgery.

• #71 Prognostic importance of numbers of retrieved lymph nodes and positive lymph nodes for ampulla of vater cancer (AVC) in 2347 patients from the Surveillance, Epidemiology, and End Results (SEER) database | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0244987

  The numbers of retrieved lymph nodes (RLNs) and positive lymph nodes (PLNs) had a significant impact on the survival of patients with ampulla of vater cancer (AVC), but the optimal numbers of the both were controversial. […] The study found that the optimal cut-off values of RLNs were 18 in the N0 cohort and 16 in N1 or entire cohort due to the highest 5-year overall survival (OS) rate and disease-specific survival (DSS) rate and the separation of survival curves (all P 0.05). […] This article recommended that at least 16 lymph nodes will improve the prognosis of AVC patients undergoing surgery. The best cut-off values of PLNs recommended for this study were 0 and 2, which may accurately stratify patients. […] The optimal cut-off value of RLNs supported by this study was 16. Adequate lymph node dissection may improve the prognosis of patients undergoing radical surgery for malignant tumors. Thus, routine lymph node dissection was recommended in AVC patients who underwent surgery.

• #72 Prognostic importance of numbers of retrieved lymph nodes and positive lymph nodes for ampulla of vater cancer (AVC) in 2347 patients from the Surveillance, Epidemiology, and End Results (SEER) database | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0244987

  The best cut-off values of PLNs recommended for this study were 0 and 2. Although there were many predictors affecting the prognosis of AVC, such as age, tumor size, and differentiation, lymph node status was recognized as a key factor affecting the prognosis of the patients. […] This study recommended that at least 16 lymph nodes will improve the prognosis of patients with AVC undergoing surgery. The best cut-off values of PLNs recommended for this study were 0 and 2, which may accurately stratify patients.

• #73 Prognostic importance of numbers of retrieved lymph nodes and positive lymph nodes for ampulla of vater cancer (AVC) in 2347 patients from the Surveillance, Epidemiology, and End Results (SEER) database | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0244987

  The best cut-off values of PLNs recommended for this study were 0 and 2. Although there were many predictors affecting the prognosis of AVC, such as age, tumor size, and differentiation, lymph node status was recognized as a key factor affecting the prognosis of the patients. […] This study recommended that at least 16 lymph nodes will improve the prognosis of patients with AVC undergoing surgery. The best cut-off values of PLNs recommended for this study were 0 and 2, which may accurately stratify patients.

• #74 Proposal of a new T-stage classification system for ampullary carcinoma based on Surveillance, Epidemiology and End Result (SEER) database

  http://www.hbpdint.com/EN/abstract/abstract5222.shtml

  Proposal of a new T-stage classification system for ampullary carcinoma based on Surveillance, Epidemiology and End Result (SEER) database […] Using the Surveillance, Epidemiology, and End Results (SEER) database, we identified 1080 patients with ampullary carcinoma who underwent radical surgical resection between 2004 and 2015. […] The new T-stage classification system described herein can better differentiate prognostic outcomes after radical resection in patients with ampullary carcinoma by incorporating tumor size and depth of tumor infiltration.

• #75 Proposal of a new T-stage classification system for ampullary carcinoma based on Surveillance, Epidemiology and End Result (SEER) database

  http://www.hbpdint.com/EN/abstract/abstract5222.shtml

  Proposal of a new T-stage classification system for ampullary carcinoma based on Surveillance, Epidemiology and End Result (SEER) database […] Using the Surveillance, Epidemiology, and End Results (SEER) database, we identified 1080 patients with ampullary carcinoma who underwent radical surgical resection between 2004 and 2015. […] The new T-stage classification system described herein can better differentiate prognostic outcomes after radical resection in patients with ampullary carcinoma by incorporating tumor size and depth of tumor infiltration.

• #76 Nomogram for overall survival in ampullary adenocarcinoma using the surveillance, epidemiology, and end results database and external validation

  https://www.wjgnet.com/2218-4333/full/v16/i2/95910-g005.htm

  Nomogram for overall survival in ampullary adenocarcinoma using the surveillance, epidemiology, and end results database and external validation. […] Yang J, Wang ZY, Chen J, Zhang Y, Chen L. Nomogram for overall survival in ampullary adenocarcinoma using the surveillance, epidemiology, and end results database and external validation. World J Clin Oncol 2025; 16(2): 95910.

• #77 Biliary Tract Cancers: Bile Duct, Gallbladder, and Ampulla of Vater – NCI

  https://dceg.cancer.gov/research/cancer-types/biliary-tract-bile-duct/biliary-tract-cancers

  To better understand the etiology of rare cancers that form in the biliary tract (bile duct, gallbladder, ampulla of Vater), DCEG researchers are conducting studies to examine the molecular and epidemiologic characteristics of cancer at different sites in the biliary tract and identify potential molecular subtypes. […] As more samples and accompanying exposure information are collected, investigators are able to study increasingly rare subtypes with cutting-edge techniques to determine the epidemiologic and molecular contributors to the development of biliary tract cancers. […] The Shanghai Biliary Tract Case-Control Study has the primary goal of investigating biliary tract cancers, including investigation of biologic markers and epidemiologic risk factors for gallbladder, extrahepatic bile duct, and ampulla of Vater cancers.

• #78 Biliary Tract Cancers: Bile Duct, Gallbladder, and Ampulla of Vater – NCI

  https://dceg.cancer.gov/research/cancer-types/biliary-tract-bile-duct/biliary-tract-cancers

  To better understand the etiology of rare cancers that form in the biliary tract (bile duct, gallbladder, ampulla of Vater), DCEG researchers are conducting studies to examine the molecular and epidemiologic characteristics of cancer at different sites in the biliary tract and identify potential molecular subtypes. […] As more samples and accompanying exposure information are collected, investigators are able to study increasingly rare subtypes with cutting-edge techniques to determine the epidemiologic and molecular contributors to the development of biliary tract cancers. […] The Shanghai Biliary Tract Case-Control Study has the primary goal of investigating biliary tract cancers, including investigation of biologic markers and epidemiologic risk factors for gallbladder, extrahepatic bile duct, and ampulla of Vater cancers.

• #79 Biliary Tract Cancers: Bile Duct, Gallbladder, and Ampulla of Vater – NCI

  https://dceg.cancer.gov/research/cancer-types/biliary-tract-bile-duct/biliary-tract-cancers

  To better understand the etiology of rare cancers that form in the biliary tract (bile duct, gallbladder, ampulla of Vater), DCEG researchers are conducting studies to examine the molecular and epidemiologic characteristics of cancer at different sites in the biliary tract and identify potential molecular subtypes. […] As more samples and accompanying exposure information are collected, investigators are able to study increasingly rare subtypes with cutting-edge techniques to determine the epidemiologic and molecular contributors to the development of biliary tract cancers. […] The Shanghai Biliary Tract Case-Control Study has the primary goal of investigating biliary tract cancers, including investigation of biologic markers and epidemiologic risk factors for gallbladder, extrahepatic bile duct, and ampulla of Vater cancers.

• #80 Risk of subsequent primary cancers in patients with carcinoma of the Ampulla of Vater | British Journal of Cancer

  https://www.nature.com/articles/bjc1997539

  Data were collected on subsequent primary cancers occurring in 194 individuals diagnosed with ampullary carcinoma during 1979-92 in the north western region of England, UK. Four cancers were identified compared with 6.62 expected (relative risk 0.60), suggesting that individuals with ampullary carcinoma are not at increased risk of developing subsequent primary cancers.

• #81 The Diagnosis and Treatment of Ampullary Carcinoma (27.10.2023)

  https://di.aerzteblatt.de/int/archive/article/234965

  Ampullary carcinoma has an incidence of 0.5 to 0.9 per 100 000 persons and a poor prognosis, with a 5-year survival rate of 41% to 45% for locally confined and 4% to 7% for metastatic disease. […] The ICD-10 code for ampullary carcinomas is C24.1; the cancer data from the RKI have to date not reported a separate incidence. For extrahepatic cholangiocarcinomas (together with C24.0, C22.8, and C22.9) the incidence in Germany in 2015 was 2.9/100 000 in men and 1.8/100 000 in women. […] Five-year survival rates varied between 10% and 75% in the localized stage and 4.7% in the metastatic stage. […] The treatment of choice in ampullary carcinoma is radical oncological resection of the head of the pancreas with systematic lymphadenectomy. […] The poor prognosis even at the early resectable stage prompts the question of whether adjuvant therapeutic concepts can potentially improve the disease course.

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