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Scandinavian Journal of Gastroenterology 2018Duodenal neuroendocrine neoplasms (dNENs) are rare tumors, which usually show good prognosis. The optimal management of these tumors is still far from being clearly... (Review)
Review
BACKGROUND
Duodenal neuroendocrine neoplasms (dNENs) are rare tumors, which usually show good prognosis. The optimal management of these tumors is still far from being clearly understood because of their rarity and the poor level of knowledge about their natural history. Herein, we have reviewed the literature on dNENs to collect and analyze the current data on epidemiology, diagnosis and management of these rare tumors.
METHODS
Bibliographical searches were performed in PubMed, using the following keywords: duodenal neuroendocrine neoplasm; duodenum; gastrinoma; diagnosis; therapy; guidelines. We searched for all relevant articles published over the last 15 years. Non-English language papers were excluded.
RESULTS
We reviewed the pertinent articles about dNENs. Upper gastrointestinal endoscopy with biopsy is the cornerstone of the dNENs diagnostic process. Endoscopic ultrasound with fine-needle aspiration/biopsy should be performed in order to locally stage the disease and in all cases of non-diagnostic endoscopy. Endoscopic or complete surgical removal of the primary lesion is the recommended treatment and is generally achievable for the majority of the patients. A less aggressive approach may be suggested for well-differentiated low-stage tumors. After NEN removal, patients should be closely followed-up especially during the first 3 years by endoscopic examination, imaging tests and CgA measurements.
CONCLUSIONS
The multi-disciplinary approach and the preservation of the quality of life of the patients play a key role in the therapeutic process for dNENs. Further studies are needed to better define standardized guidelines specific to dNENs, including optimal management approaches and follow-up intervals.
Topics: Duodenal Neoplasms; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Endoscopy, Digestive System; Gastrinoma; Humans; Neuroendocrine Tumors; Practice Guidelines as Topic; Quality of Life
PubMed: 29726295
DOI: 10.1080/00365521.2018.1468479 -
Journal of Surgical Oncology Feb 2021Periampullary neuroendocrine tumors (NETs) arise from the duodenum, ampulla, and periampullary pancreas. Duodenal and ampullary NETs are rare and may have distinct...
BACKGROUND
Periampullary neuroendocrine tumors (NETs) arise from the duodenum, ampulla, and periampullary pancreas. Duodenal and ampullary NETs are rare and may have distinct biologic behavior from pancreatic NETs (P-NETs). We examined the outcomes of these entities.
METHODS
An institutional database was queried for patients undergoing resection for pancreatic head, duodenal, or ampullary NETs from 2000 to 2018. Patients with MEN1 syndrome or follow up less than 12 months were excluded.
RESULTS
Three hundred and ten patients were identified. Tumor locations were ampulla (n = 15), duodenum (n = 35) and pancreas (n = 260). Median follow-up and recurrence-free survival (RFS) were 60.9 (interquartile range [IQR]: 34.8-99.3) and 171.7 (IQR: 84.0-NR) months. Clinicopathologic data and survival outcomes were similar for duodenal and ampullary NETs (RFS: p = .347 and overall survival [OS]: p = .246) and were combined into an intestinal subtype (IS) group. There were no differences in OS or RFS when comparing IS-NET and P-NET. On multivariate analysis, tissue of origin was not associated with risk of recurrence. The current American Joint Committee on Cancer staging guidelines, which account for origin tissue, were predictive of outcomes for all subtypes.
CONCLUSION
Tissue of origin does not appear to impact long-term outcomes when comparing IS-NETs and P-NETs. The AJCC staging system offers good discriminatory capacity in the context of the tissue type.
Topics: Adult; Aged; Aged, 80 and over; Ampulla of Vater; Common Bile Duct Neoplasms; Duodenal Neoplasms; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Staging; Neuroendocrine Tumors; Pancreatic Neoplasms; Pancreaticoduodenectomy; Prospective Studies; Survival Rate; Tumor Burden
PubMed: 33125737
DOI: 10.1002/jso.26285 -
Gastrointestinal Endoscopy Feb 2021
Topics: Duodenal Diseases; Duodenal Neoplasms; Duodenum; Humans; Intestinal Polyps; Neoplasms, Glandular and Epithelial
PubMed: 33478662
DOI: 10.1016/j.gie.2020.10.010 -
Digestive Endoscopy : Official Journal... Mar 2016Historically, neoplasia of the duodenal papilla has been managed surgically, which may be associated with substantial morbidity and mortality. In the absence of invasive... (Review)
Review
Historically, neoplasia of the duodenal papilla has been managed surgically, which may be associated with substantial morbidity and mortality. In the absence of invasive cancer, even lesions with extensive lateral duodenal wall involvement, or limited intraductal extension may be cured endoscopically with a superior safety profile. Endoscopic papillectomy is associated with greater risks of adverse events such as bleeding than resection elsewhere in the gastrointestinal tract. Additionally site-specific complications such as pancreatitis exist. A structured approach to lesion assessment, adherence to technical aspects of resection, endoscopic management of complications and post-resection surveillance is required. Advances have been made in all facets of endoscopic papillary resection since its introduction in the 1980s; extending the boundaries of endoscopic cure, optimizing outcomes and enhancing patient safety. These will be the focus of the present review.
Topics: Adenoma; Duodenal Neoplasms; Endoscopy, Gastrointestinal; Humans; Neoplasm Staging
PubMed: 26573214
DOI: 10.1111/den.12574 -
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 -
Journal of Gastroenterology and... Sep 2014Safety and efficacy data on endoscopic treatment of duodenal neoplasm are limited. We suggest the technical feasibility of endoscopic procedures by evaluating the...
BACKGROUND AND AIM
Safety and efficacy data on endoscopic treatment of duodenal neoplasm are limited. We suggest the technical feasibility of endoscopic procedures by evaluating the results of endoscopic treatment for nonampullary duodenal adenoma and adenocarcinoma.
METHODS
Forty-five patients who underwent endoscopic treatment for nonampullary duodenal adenoma with or without malignant transformation between September 2003 and March 2012 were included. Endoscopic polypectomy of duodenal polyp (DPP), duodenal endoscopic mucosal resection (DEMR), and duodenal endoscopic submucosal dissection (DESD) were selected as endoscopic treatments for each lesion.
RESULTS
Mean lesion size was 9.1 mm, and most lesions were located in the second portion of the duodenum. There were 40 adenomas and five early-stage adenocarcinomas arising from adenomas. Of the 45 duodenal neoplasms, five patients were treated with DPP, 33 with DEMR, and seven patients with a large duodenal lesion underwent DESD. Minimum of 1-year follow-up endoscopies were performed in 42 patients, excepting three patients treated after October 2011. Median follow-up was 24.8 months. Of the 45 patients, en bloc resection was performed in 43 (95.6%). A complete resection was performed in 41 patients (91.1%). No significant bleeding events occurred. Perforations occurred in three patients who underwent DESD. All perforations were noticed during the procedures and completely closed by endoscopic clipping. There was one recurrence at 6 months after DPP.
CONCLUSION
Endoscopic treatment is minimally invasive management for duodenal adenomas and superficial adenocarcinomas. It would be helpful for medical doctors in the management of duodenal neoplasms.
Topics: Adenocarcinoma; Adenoma; Adult; Aged; Aged, 80 and over; Duodenal Neoplasms; Duodenoscopy; Follow-Up Studies; Humans; Male; Middle Aged; Retrospective Studies; Time; Treatment Outcome
PubMed: 24720570
DOI: 10.1111/jgh.12601 -
Mayo Clinic Proceedings Nov 2017
Topics: Adenoma; Adult; Brunner Glands; Duodenal Neoplasms; Humans; Male
PubMed: 29101941
DOI: 10.1016/j.mayocp.2017.08.018 -
The International Journal of Medical... Apr 2021The unique and complex anatomical location of duodenal juxta-ampullary neoplasms complicates selection of the appropriate surgical strategy. For benign or borderline...
BACKGROUND
The unique and complex anatomical location of duodenal juxta-ampullary neoplasms complicates selection of the appropriate surgical strategy. For benign or borderline tumours, surgical local resection can be an appropriate treatment option, and robotic surgical systems can help perform minimally invasive local resection of these lesions.
METHODS
Between December 2014 and December 2019, 10 patients who underwent robotic local resections for duodenal juxta-ampullary tumours were reviewed.
RESULTS
All patients successfully underwent robotic local resection of the duodenum, preserving the ampulla of Vater without conversion. The mean tumour size was 2.2 cm. Final pathology consisted of gastrointestinal stromal tumour, neuroendocrine tumour, low grade and high grade dysplasia, ectopic pancreas, and well-differentiated adenocarcinoma (T1a). There were no postoperative complications or recurrences.
CONCLUSION
With accurate preoperative diagnosis and careful selection of patients, local resection of the duodenum for juxta-ampullary benign or borderline tumours using robotic surgical system is an attractive treatment option.
Topics: Ampulla of Vater; Common Bile Duct Neoplasms; Duodenal Neoplasms; Humans; Pancreatic Neoplasms; Retrospective Studies; Robotic Surgical Procedures
PubMed: 33125798
DOI: 10.1002/rcs.2192 -
Clinical Colorectal Cancer Dec 2019Small bowel adenocarcinomas (SBAs) are rare tumors. Management of SBA is extrapolated from colorectal cancer treatments. Recent evidence suggests that the biology and...
BACKGROUND
Small bowel adenocarcinomas (SBAs) are rare tumors. Management of SBA is extrapolated from colorectal cancer treatments. Recent evidence suggests that the biology and molecular features of SBA differ from colorectal cancer. The aim of this study was to evaluate the management and outcome of SBA patients. PATIENTS AND METHODS: The National Cancer Data Base (NCDB) was queried for patients with SBA between 2004 and 2013 using ICD-O-3 histology code 8140/3 and topography codes C17.0, C17.1, C17.2, C17.8, and C17.9. Univariate and multivariate survival analyses were conducted to analyze the association between SBA location and overall survival (OS) stratified by stage. Treatment outcomes of surgery, radiation, and systemic therapy were compared.
RESULTS
A total of 7954 SBA patients were identified; duodenum (D) 4607 (57.9%), jejunum (J) 1241 (15.6%), ileum (I) 857 (10.8%), and unspecified 1249 (15.7%). A total of 53.6% patients were male, and 76.6% white. Median age was 66 years. D mostly presented as stage IV disease (37.6%), J as stage II (34.5%) and IV disease (33.8%), and I as stage II (32.2%) and III (30.3%) disease (P < .001). Grade distribution was similar among D, J, and I; the majority were moderately differentiated (40.8%-55.0%), followed by poorly differentiated (30.9%-35.8%) and well differentiated (6.0%-12.4%) (P < .001). D underwent surgery (50.2%) less often than J (90.8%) and I (94.5%) (P < .001). Adjuvant radiation was provided in 8.5% of D, 2.6% of J, and 2.1% of I (P < .001). Adjuvant chemotherapy was provided in 21.9% of D, 50.2% of J, and 42.0% of I (P < .001). The rate of adjuvant chemotherapy was the highest in patients with stage III SBA, and was as follows: D (43.4%), J (65.4%), and I (63.6%) (P < .001). In univariate and multivariate analyses of all patients, adjuvant chemotherapy was associated with improved OS in stage II-III SBA patients. J had the best 5-year OS rate (42.0%; 95% confidence interval, 38.8-45.1, P < .001), and D had the worst (23.0%; 95% confidence interval, 21.6-24.2, P < .001). In multivariate analysis stratified by stage, chemotherapy was associated with improved OS in patients with stage II-IV SBA.
CONCLUSION
Most SBA patients present with stage IV disease. D underwent surgery less often than J and I. Stage II and III D received adjuvant chemotherapy less often compared to stage II and III J and I. Adjuvant chemotherapy was associated with improved OS in patients with stage II-III disease. J had the best 5-year OS rate, and D had the worst.
Topics: Adenocarcinoma; Adolescent; Adult; Aged; Aged, 80 and over; Combined Modality Therapy; Duodenal Neoplasms; Female; Follow-Up Studies; Humans; Ileal Neoplasms; Jejunal Neoplasms; Male; Middle Aged; Neoplasm Invasiveness; Prognosis; Retrospective Studies; Survival Rate; Young Adult
PubMed: 31606297
DOI: 10.1016/j.clcc.2019.08.002 -
Der Chirurg; Zeitschrift Fur Alle... Apr 2016Neuroendocrine neoplasms (NEN) represent a group of potentially malignant tumors, which can be located in every section of the gastrointestinal tract, the pancreas and... (Review)
Review
Neuroendocrine neoplasms (NEN) represent a group of potentially malignant tumors, which can be located in every section of the gastrointestinal tract, the pancreas and the bronchopulmonary system. Gastroduodenal NENs have a relatively good prognosis in comparison to other subentities, e.g. pancreatic or ileojejunal NENs. In the stomach there are four different types of NENs, while in the duodenum there are five types and all vary in the malignant potential and the therapeutic approach. Due to the simple access endoscopic methods not only have diagnostic but also important therapeutic relevance in this subgroup. Lesions smaller than 1 cm can easily be resected with forceps or snare resection and for larger lesions up to 2 cm more invasive strategies, such as endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD) are available. Important criteria in gastric NEN for the risk evaluation of endoscopic treatment alone are the size of the lesion, depth of invasion and the tumor biology, e.g. neuroendocrine tumor (NET) G1/G2 versus neuroendocrine carcinoma (NEC) G3. In duodenal NEN the aforementioned risk factors also apply and in addition only lesions outside the ampulla of Vater should be endoscopically resected whereas periampullary lesions need to be addressed surgically. As an individualized therapeutic approach the possibility of a combined endoscopic and laparoscopic resection technique exists. Follow-up endoscopic investigations are necessary, especially in gastric type 1 NENs, which have a tendency to relapse.
Topics: Duodenal Neoplasms; Duodenoscopy; Gastroscopy; Humans; Neoplasm Grading; Neoplasm Invasiveness; Neoplasm Staging; Prognosis; Risk Factors; Stomach Neoplasms; Zollinger-Ellison Syndrome
PubMed: 26960437
DOI: 10.1007/s00104-016-0157-6