-
American Society of Clinical Oncology... 2014Ampullary cancers are rare, accounting for only 0.2% of gastrointestinal cancers and approximately 7% of all periampullary cancers. They arise from the ampullary... (Review)
Review
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 (Fig. 1). 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. Herein, we provide an overview of the biology, histology, current therapeutic strategies, and potential future therapies for carcinomas arising from the ampulla of Vater.
Topics: Adenocarcinoma; Ampulla of Vater; Chemoradiotherapy, Adjuvant; Common Bile Duct Neoplasms; Humans; Neoadjuvant Therapy; Neoplasm Recurrence, Local; Neoplasm Staging; Pancreaticoduodenectomy; Radiotherapy, Adjuvant; Risk Factors; Time Factors; Treatment Outcome
PubMed: 24857067
DOI: 10.14694/EdBook_AM.2014.34.112 -
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 -
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 -
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 -
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 -
Deutsches Arzteblatt International Oct 2023Ampullary or papillary carcinoma is a malignant tumor arising from the mucosa in the region of the major duodenal papilla, also known as the ampulla of Vater. Uniform... (Review)
Review
BACKGROUND
Ampullary or papillary carcinoma is a malignant tumor arising from the mucosa in the region of the major duodenal papilla, also known as the ampulla of Vater. Uniform treatment recommendations are lacking both for the adjuvant situation and for palliative care.
METHODS
A selective literature search was carried out in PubMed in order to identify the most informative publications concerning the epidemiology, clinico-pathological background, and surgical and medical treatment of this condition.
RESULTS
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. Most such tumors are of an intestinal or a pan - creaticobiliary immunohistochemical subtype; the latter has a worse prognosis (median survival, 72-80 vs. 33-41 months). Targeted treatment is not yet available for either subtype, nor is there enough scientific evidence available for the formulation of specific therapeutic recommendations in either the adjuvant or the palliative situation. The treatment of choice for ampullary carcinoma is radical oncological resection of the head of the pancreas with systematic lymphadenectomy. Five-year overall survival is between 10% and 75% depending on the stage. No definitive recommendation for adjuvant therapy can be given. Palliative therapy can be oriented to the published treatment strategies for cancer of the colon, pancreas, and bile duct.
CONCLUSION
The current state of the evidence on the treatment of ampullary carcinoma is poor. Therapeutic decisions should be discussed in an interdisciplinary tumor board and should, in our opinion, take the histological subtype into account.
Topics: Humans; Ampulla of Vater; Adenocarcinoma; Prognosis; Pancreatic Neoplasms; Combined Modality Therapy
PubMed: 37656482
DOI: 10.3238/arztebl.m2023.0195 -
Cancer Medicine Jul 2019There is no standard salvage chemotherapy for metastatic periampullary adenocarcinoma and duodenal adenocarcinoma and the prognosis of those who fail oxaliplatin,...
BACKGROUND/AIM
There is no standard salvage chemotherapy for metastatic periampullary adenocarcinoma and duodenal adenocarcinoma and the prognosis of those who fail oxaliplatin, irinotecan, and 5FU is dismal. We examined nanoparticle albumin-bound paclitaxel (nab-paclitaxel) as salvage therapy for these two malignancies.
METHODS
Patients who failed oxaliplatin, irinotecan, and 5FU and whose archival tumors stained immunohistochemical (IHC) tumor positive for CK7 or MUC1 received nab-paclitaxel and gemcitabine therapy with or without cisplatin.
RESULTS
Three patients, 2 with metastatic ampullary adenocarcinoma and 1 with duodenal adenocarcinoma with positive IHC staining for CK7 or MUC1 who failed 2 lines of chemotherapy with oxaliplatin, irinotecan, and 5FU received nab-paclitaxel and gemcitabine with or without cisplatin. All achieved excellent tumor response on CT scans with marked falls in tumor markers CA19-9 and CEA as well as ≥1 year of progression-free survival. All 3 have continued to survive 2-3 years since diagnosed with stage 4 metastatic adenocarcinoma.
CONCLUSIONS
Nab-paclitaxel plus gemcitabine with or without cisplatin should be investigated as a standard-of-care chemotherapy regimen for patients with ampullary adenocarcinoma and duodenal adenocarcinoma.
Topics: Adenocarcinoma; Albumins; Ampulla of Vater; Antineoplastic Combined Chemotherapy Protocols; Biomarkers; Deoxycytidine; Duodenal Neoplasms; Female; Humans; Immunohistochemistry; Male; Neoplasm Metastasis; Neoplasm Staging; Paclitaxel; Tomography, X-Ray Computed; Treatment Outcome; Gemcitabine
PubMed: 31102323
DOI: 10.1002/cam4.2181 -
Diagnostic and Interventional Imaging Oct 2017Recent advances in imaging have resulted in marked changes in the investigation of the duodenum, which still remains primarily evaluated with videoendoscopy. However,... (Review)
Review
Recent advances in imaging have resulted in marked changes in the investigation of the duodenum, which still remains primarily evaluated with videoendoscopy. However, improvements in computed tomography (CT) and magnetic resonance (MR) imaging have made detection and characterization of duodenal mass-forming abnormalities easier. The goal of this pictorial review was to illustrate the most common conditions of the duodenum that present as mass-forming lesions with a specific emphasis on CT and MR imaging. MR imaging used in conjunction with duodenal distension appears as a second line imaging modality for the characterization of duodenal mass-forming lesions. CT remains the first line imaging modality for the detection and characterization of a wide range of duodenal mass-forming lesions.
Topics: Adenocarcinoma; Adenoma; Arteriovenous Malformations; Brunner Glands; Choristoma; Diverticulum; Duodenal Diseases; Duodenum; Gastrointestinal Stromal Tumors; Hamartoma; Hemangioma; Humans; Hyperplasia; Intestinal Polyposis; Leiomyoma; Lipoma; Lymphoma; Magnetic Resonance Imaging; Neuroendocrine Tumors; Pancreas; Tomography, X-Ray Computed; Tuberculosis, Gastrointestinal
PubMed: 28185840
DOI: 10.1016/j.diii.2017.01.004 -
California Medicine Sep 1963Carcinoid tumors are diminutive growths found along the gastrointestinal tract, in the lungs and, occasionally, within the confines of teratomas. Carcinoid tumors of the...
Carcinoid tumors are diminutive growths found along the gastrointestinal tract, in the lungs and, occasionally, within the confines of teratomas. Carcinoid tumors of the intestinal tract are found in decreasing order of frequency in the rectum, the appendix, the ileum, the jejunum, the lung, the stomach and the duodenum. Arising from argentaffin elements in the crypts of Lieberkuehn, and secreting serotonin, carcinoid tumors belong to the order of functioning neoplasms. Their indolent growth seems to place them between benign neoplasms and carcinomas. However, they possess propensities for metastasis that correlate with increase in size. The symptoms that are produced are almost always due to the presence of metastatic lesions. In ileal and gastric carcinoid tumors, the tendency to metastasis is augmented and the metastatic masses are sometimes of sufficient bulk to cause the carcinoid syndrome or to interfere with the supply of blood to the affected segment. More often they produce the clinical picture of intestinal obstruction. The unusually long interval from onset to death associated with carcinoid tumors makes palliative subtotal resections and short-circuiting operations in symptomatic patients with advanced disease worthwhile, for by such unorthodox procedures the patients may be afforded many additional years of useful life.
Topics: Appendiceal Neoplasms; Appendix; Bronchial Neoplasms; Carcinoid Tumor; Carcinoma, Neuroendocrine; Duodenum; Humans; Intestinal Neoplasms; Jejunum; Malignant Carcinoid Syndrome; Rectal Neoplasms; Rectum; Stomach Neoplasms
PubMed: 14047626
DOI: No ID Found