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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 -
The American Surgeon Oct 2014Adenocarcinoma of the small bowel accounts for only one per cent of all gastrointestinal malignancies. Duodenal adenocarcinoma accounts for half of all small bowel... (Review)
Review
Adenocarcinoma of the small bowel accounts for only one per cent of all gastrointestinal malignancies. Duodenal adenocarcinoma accounts for half of all small bowel adenocarcinomas. The duodenum is divided into four segments: D1 (proximal horizontal 5 cm beginning with the 3-cm duodenal bulb), D2 (descending), D3 (distal horizontal), and D4 (ascending). The most common location of duodenal adenocarcinomas is the ampullary region of D2. Based on observational experience, our hypothesis was that primary adenocarcinomas arising from the mucosa of the duodenal bulb are extremely rare or possibly nonexistent. Our institutional cancer registry provided a list of patients for the years 1990 through 2012 who had small bowel cancers. Only those patients with primary adenocarcinomas of the duodenal mucosa were reviewed. Ampullary cancers arising from bile duct mucosa were specifically excluded. Medical records were abstracted to obtain patient age, sex, race, anatomic location of the tumor, disease stage (as per American Joint Committee on Cancer 7th edition staging guidelines), operation performed, and current vital status. A total of 30 patients with primary duodenal adenocarcinomas were identified. The mean age was 58 years and 17 (57%) patients were male. The tumor locations were: D2 in 26 (87%), D3 in two (7%), and D4 in two (7%). No tumors arose from D1. The patients presented with the following stages of disease: Stage 0is in three (10%), Stage I in three (10%), Stage II in five (17%), Stage III in 15 (50%), and Stage IV in four (13%). These findings combined with a diligent review of 724 reported cases in the English language literature yielded only five clearly defined cases of adenocarcinoma arising from the mucosa of the duodenal bulb. Although a 1991 published multicenter tumor registry series of 128 localized duodenal adenocarcinomas reported 29 D1 tumors, no anatomic distinction was made between duodenal bulb and more distal D1 tumors. Earlier reports used nonanatomic divisions of the duodenum or a simple breakdown into supra-ampullary, periampullary, and infra-ampullary portions. These data beg the question as to why primary duodenal bulb adenocarcinomas are so exceedingly rare. The obvious implication is that the duodenal bulb mucosa may be physiologically, immunologically, or otherwise uniquely privileged to virtually escape oncogenic transformation. The scientific challenge and opportunity is to explore and understand the important phenomena responsible for this finding.
Topics: Adenocarcinoma; Duodenal Neoplasms; Duodenum; Female; Humans; Intestinal Mucosa; Male; Middle Aged; Neoplasm Staging; Registries
PubMed: 25264638
DOI: No ID Found -
Der Chirurg; Zeitschrift Fur Alle... Sep 2021Approximately 30-40% of pancreatoduodenectomies for adenocarcinomas result in nonpancreatic periampullary adenocarcinoma as the final diagnosis. Depending on the origin,... (Review)
Review
BACKGROUND
Approximately 30-40% of pancreatoduodenectomies for adenocarcinomas result in nonpancreatic periampullary adenocarcinoma as the final diagnosis. Depending on the origin, a distinction is made between four different carcinomas with histomorphological subtypes.
OBJECTIVE
Carcinoma location and subtype are of prognostic and therapeutic relevance; however, the preoperative differentiation is often incorrect despite modern diagnostics.
MATERIAL AND METHODS
Overview of the current literature on the classification and preoperative diagnostics of periampullary adenocarcinomas.
RESULTS
A precise knowledge of the papillary anatomy is necessary for the correct classification of diagnostic findings. Current studies demonstrate diagnostically valuable information from the anamnesis, imaging and endoscopy.
CONCLUSION
In ca. 70-80% of cases a correct diagnosis of the type of periampullary adenocarcinoma is possible on the basis of interdisciplinary diagnostics. This potentially enables a correspondingly individualized treatment planning in the preoperative phase.
Topics: Adenocarcinoma; Ampulla of Vater; Common Bile Duct Neoplasms; Duodenal Neoplasms; Humans; Pancreatic Neoplasms; Pancreaticoduodenectomy
PubMed: 34264368
DOI: 10.1007/s00104-021-01452-3 -
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 -
Der Chirurg; Zeitschrift Fur Alle... Sep 2021Ampullary carcinoma is a rare malignant neoplasm and arises in the region of Vater's ampulla. The differentiation from pancreatic and distal cholangiocarcinoma can be... (Review)
Review
Ampullary carcinoma is a rare malignant neoplasm and arises in the region of Vater's ampulla. The differentiation from pancreatic and distal cholangiocarcinoma can be difficult. The prognosis is more favorable than for pancreatic ductal adenocarcinoma but recurrences are frequent. An exact diagnostic clarification and differentiation from pancreatic carcinoma is therefore essential. Although the resection of periampullary carcinoma is established, prospectively controlled studies on the role of multimodal treatment are rare. Adjuvant chemotherapy is oriented to the protocols for pancreatic carcinoma and could be of benefit in lymph node metastases, advanced T stage and low differentiation of tumors. Intestinal and pancreatobiliary subtypes can be differentiated histologically, which is relevant for systemic treatment strategies. Patients with pancreatobiliary differentiated tumors in particular could benefit from gemcitabine-based treatment but insufficient evidence exists for chemoradiotherapy. The role of neoadjuvant and perioperative treatment strategies is currently unclear. Molecular characterization can help to identify familial risk constellations and targeted treatment strategies for this rare tumor entity.
Topics: Adenocarcinoma; Ampulla of Vater; Bile Duct Neoplasms; Bile Ducts, Intrahepatic; Combined Modality Therapy; Common Bile Duct Neoplasms; Humans; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Prognosis
PubMed: 34228145
DOI: 10.1007/s00104-021-01454-1 -
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 -
Journal of Gastrointestinal Cancer Mar 2023Small intestinal cancers have a non-specific clinical presentation and hence a delayed diagnosis. The prevalence of small intestinal cancers is low, and there are no...
PURPOSE
Small intestinal cancers have a non-specific clinical presentation and hence a delayed diagnosis. The prevalence of small intestinal cancers is low, and there are no cost-effective methods of screening. This study aimed to identify clinical characteristics of duodenal and jejunal adenocarcinomas that can assist in the early detection and diagnosis of disease.
METHODS
Duodenal adenocarcinoma and jejunal adenocarcinoma in Explorys database (1999-2019) were compared using odds ratio (OR) with 95% confidence intervals. Data on demographic characteristics, risk factors, clinical features, and treatment were collected.
RESULTS
Out of a total of 8100 patients with a diagnosis of primary adenocarcinoma of the small intestine, 5110 are primary adenocarcinoma of duodenum (63%), and 600 are primary adenocarcinoma of jejunum (7.4%). Patients with jejunal adenocarcinoma when compared with patients with duodenal adenocarcinoma are more obese (OR, 1.36) and have a significantly higher prevalence of malignant neoplasm of colon (OR, 3.07), Crohn's disease (OR, 4.42), and celiac disease (OR, 2.48). Jejunal adenocarcinoma patients presented more frequently with intestinal obstruction (OR, 1.99), whereas duodenal adenocarcinoma patients more commonly presented with iron deficiency anemia (OR, 0.16). Patients with jejunal adenocarcinoma are less likely to undergo therapy with anti-neoplastic agents when compared with duodenal adenocarcinoma (OR, 0.81). There are no differences in patients undergoing surgical intervention or a combination of surgical intervention and antineoplastic therapy.
CONCLUSIONS
Jejunal adenocarcinoma is more commonly associated with colorectal cancer, celiac disease, and Crohn's disease. They also had lower odds of requiring chemotherapeutic agents.
Topics: Humans; Crohn Disease; Celiac Disease; Intestine, Small; Jejunal Neoplasms; Duodenal Neoplasms; Ileal Neoplasms; Adenocarcinoma
PubMed: 35001295
DOI: 10.1007/s12029-021-00653-7 -
Seminars in Oncology Feb 2015Malignant biliary obstruction, duodenal, and gastric outlet obstruction, and tumor-related pain are the complications of unresectable pancreatic adenocarcinoma that most... (Review)
Review
Malignant biliary obstruction, duodenal, and gastric outlet obstruction, and tumor-related pain are the complications of unresectable pancreatic adenocarcinoma that most frequently require palliative intervention. Surgery involving biliary bypass with or without gastrojejunostomy was once the mainstay of treatment in these patients. However, advances in non-operative techniques-most notably the widespread availability of endoscopic biliary and duodenal stents-have shifted the paradigm of treatment away from traditional surgical management. Questions regarding the efficacy and durability of endoscopic stents for biliary and gastric outlet obstruction are reviewed and demonstrate high rates of therapeutic success, low rates of morbidity, and decreased cost. Surgery remains an effective treatment modality, and still produces the most durable relief in appropriately selected patients.
Topics: Biliary Tract Surgical Procedures; Carcinoma, Pancreatic Ductal; Duodenum; Endoscopy, Digestive System; Gastric Outlet Obstruction; Humans; Laparoscopy; Palliative Care; Pancreatic Neoplasms
PubMed: 25726060
DOI: 10.1053/j.seminoncol.2014.12.014 -
Surgical Oncology Sep 2022Most tumours in the head of the pancreas are adenocarcinomas of the exocrine pancreas. However, carcinomas located in the head of the pancreas may originate from the... (Review)
Review
Most tumours in the head of the pancreas are adenocarcinomas of the exocrine pancreas. However, carcinomas located in the head of the pancreas may originate from the papilla of Vater, the distal part of the common bile duct, or the duodenum. Tumours of that region, within 2 cm of the greater duodenal papilla, have been usually described as periampullary neoplasms. Adenocarcinomas separated from the major duodenal papilla and located in the major pancreatic duct, common bile duct, or duodenum are identified as ductal pancreatic carcinomas, distal bile duct cholangiocarcinomas or duodenal carcinomas. Surgical treatment is the only chance for cure. Pancreatoduodenectomy is the procedure of choice. Regional lymphadenectomy and removal of at least 16 lymph nodes are necessary for optimal long-term outcomes. Indications for adjuvant chemotherapy remain controversial. This review evaluates the available data on the pathological assessment of periampullary tumours and discusses the controversies of therapeutic management, emphasising adjuvant treatment.
Topics: Adenocarcinoma; Ampulla of Vater; Carcinoma, Pancreatic Ductal; Common Bile Duct Neoplasms; Humans; Pancreatic Neoplasms
PubMed: 36152420
DOI: 10.1016/j.suronc.2022.101853 -
Japanese Journal of Clinical Oncology Aug 2018Non-ampullary duodenal adenocarcinoma, excluding carcinoma in the ampulla of Vater, is a rare disease. Although several prognostic factors have been reported, they...
BACKGROUND
Non-ampullary duodenal adenocarcinoma, excluding carcinoma in the ampulla of Vater, is a rare disease. Although several prognostic factors have been reported, they remain controversial due to the rarity of non-ampullary duodenal adenocarcinoma. The aims of this study were to investigate prognostic factors in patients with non-ampullary duodenal adenocarcinoma and to assess chemotherapy in patients with recurrence.
PATIENTS AND METHODS
Records of 25 patients who underwent surgical treatment for non-ampullary duodenal adenocarcinoma from 2004 to 2016 were retrospectively reviewed. The relationship between the clinicopathological factors and outcomes was investigated.
RESULTS
Serum level of CA19-9, gross appearance, tumor size, tumor invasion, lymph node metastases, TNM stage and lymphatic and vascular invasion were significant risk factors of recurrence. Patients with recurrence who received chemotherapy according to regimens used to treat colorectal cancer had a better prognosis than those without chemotherapy (P = 0.016).
CONCLUSION
Advanced non-ampullary duodenal adenocarcinoma has a poor prognosis, but chemotherapy possibly improves the prognosis in the patients with recurrent non-ampullary duodenal adenocarcinoma.
Topics: Adenocarcinoma; Ampulla of Vater; CA-19-9 Antigen; Duodenal Neoplasms; Female; Humans; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Risk Factors; Survival Analysis
PubMed: 29931295
DOI: 10.1093/jjco/hyy086