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Journal of the National Comprehensive... Jul 2023Ampullary 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...
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. Systemic therapy is used in all stages of ampullary cancer, including neoadjuvant therapy, adjuvant therapy, and first-line or subsequent-line therapy for locally advanced, metastatic, and recurrent disease. Radiation therapy may be used in localized ampullary cancer, sometimes in combination with chemotherapy, but there is no high-level evidence to support its utility. Select tumors may be treated surgically. This article describes NCCN recommendations regarding management of ampullary adenocarcinoma.
Topics: Humans; Ampulla of Vater; Common Bile Duct Neoplasms; Duodenal Neoplasms; Adenocarcinoma; Pancreatic Neoplasms
PubMed: 37433437
DOI: 10.6004/jnccn.2023.0034 -
Current Oncology (Toronto, Ont.) Sep 2021Ampullary carcinomas (ACs) represent a rare entity, accounting for approximately 0.2% of all gastrointestinal solid tumors and 20% of all periampullary cancers (PACs).... (Review)
Review
Ampullary carcinomas (ACs) represent a rare entity, accounting for approximately 0.2% of all gastrointestinal solid tumors and 20% of all periampullary cancers (PACs). Unfortunately, few data are available regarding the optimal therapeutic strategy for ACs due to their rarity, and physicians frequently encounter significant difficulties in the management of these malignancies. In this review, we will provide an overview of current evidence on AC, especially focusing on biological features, histological characteristics, and available data guiding present and future therapeutic strategies for these rare, and still barely known, tumors.
Topics: Adenocarcinoma; Ampulla of Vater; Common Bile Duct Neoplasms; Humans
PubMed: 34590592
DOI: 10.3390/curroncol28050293 -
The American Journal of Surgical... Nov 2014Extra-ampullary duodenal adenocarcinomas are rare, and when studied, frequently have been grouped with jejunoileal adenocarcinomas. Nevertheless, anecdotal experiences...
Extra-ampullary duodenal adenocarcinomas are rare, and when studied, frequently have been grouped with jejunoileal adenocarcinomas. Nevertheless, anecdotal experiences suggest that these neoplasms may present 2 or more distinct phenotypes. To better characterize these neoplasms, we performed a retrospective review of 38 cases with a special focus on the morphologic and immunophenotypic characteristics and their clinicopathologic significance. Our cohort of extra-ampullary duodenal adenocarcinomas was classified on the basis of the morphologic features into gastric type (n=19, 50%), intestinal type (n=14, 37%), pancreaticobiliary type (n=2, 5%), and others (n=3, 8%). Most gastric-type adenocarcinomas (n=18, 95%) developed in the proximal duodenum, whereas the other types were located equally in the proximal and distal duodenum. Intestinal-type dysplasia was present at the periphery of 8 (57%) intestinal-type adenocarcinomas, and 8 (42%) gastric-type adenocarcinoma were associated with gastric-type dysplasia. Gastric foveolar metaplasia (n=12) and Brunner gland hyperplasia (n=10) were exclusively recognized adjacent to gastric-type adenocarcinomas. Notably, intestinal-type histology and the absence of lymph node metastasis were significantly associated with favorable disease-free survival in univariate and multivariate analyses. In summary, this study demonstrated that 2 major subsets of extra-ampullary duodenal adenocarcinoma, intestinal type and gastric type, are associated with distinct histopathologic features and clinical behavior.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Biopsy; Disease-Free Survival; Duodenal Neoplasms; Duodenum; Female; Humans; Immunohistochemistry; Immunophenotyping; Kaplan-Meier Estimate; Male; Metaplasia; Middle Aged; Multivariate Analysis; Phenotype; Proportional Hazards Models; Retrospective Studies; Risk Factors; Time Factors
PubMed: 25310836
DOI: 10.1097/PAS.0000000000000278 -
Monographs in Clinical Cytology 2020The pancreas is a retroperitoneal organ located in the duodenal loop with the posterior wall of the stomach overlying it and the left lobe of the liver lying anteriorly... (Review)
Review
The pancreas is a retroperitoneal organ located in the duodenal loop with the posterior wall of the stomach overlying it and the left lobe of the liver lying anteriorly to it. Tissues from these organs, in addition to the lesion of interest within the pancreas, may be sampled during fine-needle aspiration (FNA) procedures. Therefore, it is important to recognize the cytology of normal benign components of the pancreas and potential contaminants in order to render a correct diagnosis and avoid pitfalls. Normal components of the pancreas include ductal epithelial cells, acinar cells, and islet cells. In addition to the normal pancreatic cells, it is not uncommon to encounter epithelial cells from the duodenal and gastric mucosa with endoscopic ultrasound-guided fine-needle aspiration. It is important to recognize these cells as benign and to distinguish them from a well-differentiated pancreatic adenocarcinoma. Besides these, mesothelial cells and hepatocytes and bile duct cells from the liver may be sampled as well. Here, the cytological features of normal components and contaminants are described in detail.
Topics: Adenocarcinoma; Duodenum; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Gastric Mucosa; Humans; Pancreas; Pancreatic Neoplasms; Stomach
PubMed: 32987395
DOI: 10.1159/000455733 -
Archives of Pathology & Laboratory... Mar 2019Duodenal epithelial polyps are reported in 1.5% to 3% of individuals referred for upper endoscopy. Most duodenal epithelial polyps are asymptomatic and nonneoplastic;... (Review)
Review
CONTEXT.—
Duodenal epithelial polyps are reported in 1.5% to 3% of individuals referred for upper endoscopy. Most duodenal epithelial polyps are asymptomatic and nonneoplastic; however, a small subset is neoplastic and may progress to adenocarcinoma. Recent advances in immunohistochemical and molecular techniques have helped further characterize these polyps, shedding light on their origin, classification, and risk of progression to adenocarcinoma.
OBJECTIVE.—
To provide a comprehensive clinicopathologic review of nonneoplastic and neoplastic duodenal epithelial polyps, with particular emphasis on recent developments in classification schemes and risk stratification based upon immunohistochemical and molecular profiles.
DATA SOURCES.—
This review is based on peer-reviewed literature and the authors' experiences.
CONCLUSIONS.—
In this review we provide an update on the clinicopathologic, immunohistochemical, and molecular features of duodenal epithelial polyps and discuss the surveillance recommendations and treatment options available. Particular attention should be placed on recognizing duodenal adenomas with intestinal, gastric, and serrated phenotype, as they have an increased risk of malignant transformation if not completely excised.
Topics: Duodenum; Humans; Intestinal Mucosa; Intestinal Polyps
PubMed: 30354274
DOI: 10.5858/arpa.2018-0034-RA -
Nature Communications Mar 2023The subtypes of duodenal cancer (DC) are complicated and the carcinogenesis process is not well characterized. We present comprehensive characterization of 438 samples...
The subtypes of duodenal cancer (DC) are complicated and the carcinogenesis process is not well characterized. We present comprehensive characterization of 438 samples from 156 DC patients, covering 2 major and 5 rare subtypes. Proteogenomics reveals LYN amplification at the chromosome 8q gain functioned in the transmit from intraepithelial neoplasia phase to infiltration tumor phase via MAPK signaling, and illustrates the DST mutation improves mTOR signaling in the duodenal adenocarcinoma stage. Proteome-based analysis elucidates stage-specific molecular characterizations and carcinogenesis tracks, and defines the cancer-driving waves of the adenocarcinoma and Brunner's gland subtypes. The drug-targetable alanyl-tRNA synthetase (AARS1) in the high tumor mutation burden/immune infiltration is significantly enhanced in DC progression, and catalyzes the lysine-alanylation of poly-ADP-ribose polymerases (PARP1), which decreases the apoptosis of cancer cells, eventually promoting cell proliferation and tumorigenesis. We assess the proteogenomic landscape of early DC, and provide insights into the molecular features corresponding therapeutic targets.
Topics: Humans; Duodenal Neoplasms; Proteogenomics; Brunner Glands; Adenocarcinoma; Carcinogenesis
PubMed: 36991000
DOI: 10.1038/s41467-023-37221-5 -
Radiographics : a Review Publication of... 2022The duodenum can be affected by a variety of abnormalities because of its development during embryogenesis and its dual intra- and retroperitoneal location. If small...
The duodenum can be affected by a variety of abnormalities because of its development during embryogenesis and its dual intra- and retroperitoneal location. If small bowel embryogenesis is disturbed, congenital errors occur. Although some congenital variants may be asymptomatic and inconsequential to the patient, other anomalies can result in life-threatening emergencies such as malrotation, leading to midgut volvulus. Many infectious processes affect the duodenum, including duodenal ulcers and opportunistic infection in patients with HIV/AIDS or Crohn disease. Small bowel malignancies are uncommon but important to recognize, because the duodenum can be involved in polyposis syndromes or the development of primary adenocarcinoma, neuroendocrine tumors, lymphoma, and metastasis. Although endoscopy is currently the most used diagnostic method to assess the lumen of the upper gastrointestinal tract, fluoroscopy is a valuable adjunct technique and the study of choice for many diseases, specifically those for which anatomic and functional information is required. Fluoroscopy is also commonly used postoperatively to assess for complications such as obstruction and extraluminal leaks. Compared with endoscopy, fluoroscopy is an inexpensive and noninvasive technique that provides salient anatomic information and allows delineation of the duodenal mucosa and assessment of real-time duodenal motility. The authors examine the broad spectrum of conditions that can involve the duodenum, including congenital, infectious, inflammatory, and neoplastic abnormalities, and review their typical appearances at fluoroscopy. RSNA, 2022.
Topics: Duodenal Diseases; Duodenum; Fluoroscopy; Humans; Intestine, Small
PubMed: 35179986
DOI: 10.1148/rg.210165 -
The Korean Journal of Gastroenterology... Oct 2023The ampulla of Vater is a small projection formed by the confluence of the main pancreatic duct and common bile duct in the second part of the duodenum. Primary... (Review)
Review
The ampulla of Vater is a small projection formed by the confluence of the main pancreatic duct and common bile duct in the second part of the duodenum. Primary ampullary adenocarcinoma is a rare malignancy, accounting for only 0.2% of gastrointestinal cancers and approximately 7% of all periampullary cancers. Jaundice from a biliary obstruction is the most common symptom of ampullary adenocarcinoma. In the early stages, radical pancreatoduodenectomy is the standard surgical approach. On the other hand, no randomized controlled trial has provided evidence to guide physicians on the choice of adjuvant/palliative chemotherapy because of the rarity of the disease and the paucity of related research. This paper reports the biology, histology, current therapeutic strategies, and potential future therapies of ampullary adenocarcinoma.
Topics: Humans; Ampulla of Vater; Adenocarcinoma; Pancreatic Neoplasms; Common Bile Duct Neoplasms; Duodenal Neoplasms
PubMed: 37876255
DOI: 10.4166/kjg.2023.110 -
Gastroenterology Clinics of North... Sep 2016Small bowel adenocarcinomas (SBAs) are rare tumors, but their incidence is increasing. The most common primary location is the duodenum. Even though SBAs are more often... (Review)
Review
Small bowel adenocarcinomas (SBAs) are rare tumors, but their incidence is increasing. The most common primary location is the duodenum. Even though SBAs are more often sporadic, some diseases are risk factors. Early diagnosis of small bowel adenocarcinoma remains difficult, despite significant radiologic and endoscopic progress. After R0 surgical resection, the main prognostic factor is lymph node invasion. An international randomized trial (BALLAD [Benefit of Adjuvant Chemotherapy For Small Bowel Adenocarcinoma] study) will evaluate the benefit of adjuvant chemotherapy. For metastatic disease, retrospectives studies suggest that platinum-based chemotherapy is the most effective treatment. Phase II studies are ongoing to evaluate targeted therapy in metastatic SBA.
Topics: Adenocarcinoma; Adenomatous Polyposis Coli Protein; Capsule Endoscopy; Chemotherapy, Adjuvant; Digestive System Surgical Procedures; Double-Balloon Enteroscopy; Duodenal Neoplasms; Humans; Intestinal Neoplasms; Intestine, Small; Mutation; Phenotype; Prognosis; Proto-Oncogene Proteins p21(ras); Receptor, ErbB-2; Risk Factors; Tomography, X-Ray Computed; Tumor Suppressor Protein p53; beta Catenin
PubMed: 27546842
DOI: 10.1016/j.gtc.2016.04.004 -
Biomedical Papers of the Medical... Sep 2022The aim of this study was to evaluate symptoms, diagnostic methods, short- and long-term outcomes of surgical treatment in patients with duodenal adenocarcinoma. (Observational Study)
Observational Study
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.
Topics: Adenocarcinoma; Ampulla of Vater; Common Bile Duct Neoplasms; Duodenal Neoplasms; Humans; Neoplasm Staging; Pancreatic Neoplasms; Prognosis; Retrospective Studies
PubMed: 34012147
DOI: 10.5507/bp.2021.028