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World Journal of Gastroenterology Aug 2016Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, like all NETs. However, the incidence of GI-NETS has been increasing in recent years. Gastric NETs... (Review)
Review
Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, like all NETs. However, the incidence of GI-NETS has been increasing in recent years. Gastric NETs (G-NETs) and duodenal NETs (D-NETs) are the common types of upper GI-NETs based on tumor location. G-NETs are classified into three distinct subgroups: type I, II, and III. Type I G-NETs, which are the most common subtype (70%-80% of all G-NETs), are associated with chronic atrophic gastritis, including autoimmune gastritis and Helicobacter pylori associated atrophic gastritis. Type II G-NETs (5%-6%) are associated with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome (MEN1-ZES). Both type I and II G-NETs are related to hypergastrinemia, are small in size, occur in multiple numbers, and are generally benign. In contrast, type III G-NETs (10%-15%) are not associated with hypergastrinemia, are large-sized single tumors, and are usually malignant. Therefore, surgical resection and chemotherapy are generally necessary for type III G-NETs, while endoscopic resection and follow-up, which are acceptable for the treatment of most type I and II G-NETs, are only acceptable for small and well differentiated type III G-NETs. D-NETs include gastrinomas (50%-60%), somatostatin-producing tumors (15%), nonfunctional serotonin-containing tumors (20%), poorly differentiated neuroendocrine carcinomas (< 3%), and gangliocytic paragangliomas (< 2%). Most D-NETs are located in the first or second part of the duodenum, with 20% occurring in the periampullary region. Therapy for D-NETs is based on tumor size, location, histological grade, stage, and tumor type. While endoscopic resection may be considered for small nonfunctional D-NETs (G1) located in the higher papilla region, surgical resection is necessary for most other D-NETs. However, there is no consensus regarding the ideal treatment of D-NETs.
Topics: Duodenal Neoplasms; Endoscopy, Gastrointestinal; Humans; Neuroendocrine Tumors; Stomach Neoplasms
PubMed: 27570419
DOI: 10.3748/wjg.v22.i30.6817 -
Journal of Gastroenterology Dec 2022Duodenal cancer is considered to be a small intestinal carcinoma in terms of clinicopathology. In Japan, there are no established treatment guidelines based on...
Duodenal cancer is considered to be a small intestinal carcinoma in terms of clinicopathology. In Japan, there are no established treatment guidelines based on sufficient scientific evidence; therefore, in daily clinical practice, treatment is based on the experience of individual physicians. However, with advances in diagnostic modalities, it is anticipated that opportunities for its detection will increase in future. We developed guidelines for duodenal cancer because this disease is considered to have a high medical need from both healthcare providers and patients for appropriate management. These guidelines were developed for use in actual clinical practice for patients suspected of having non-ampullary duodenal epithelial malignancy and for patients diagnosed with non-ampullary duodenal epithelial malignancy. In this study, a practice algorithm was developed in accordance with the Minds Practice Guideline Development Manual 2017, and Clinical Questions were set for each area of epidemiology and diagnosis, endoscopic treatment, surgical treatment, and chemotherapy. A draft recommendation was developed through a literature search and systematic review, followed by a vote on the recommendations. We made decisions based on actual clinical practice such that the level of evidence would not be the sole determinant of the recommendation. This guideline is the most standard guideline as of the time of preparation. It is important to decide how to handle each case in consultation with patients and their family, the treating physician, and other medical personnel, considering the actual situation at the facility (and the characteristics of the patient).
Topics: Humans; Duodenal Neoplasms; Endoscopy; Japan; Neoplasms, Glandular and Epithelial
PubMed: 36260172
DOI: 10.1007/s00535-022-01919-y -
Neuroendocrinology 2021The better understanding of the biological behavior of multiple endocrine neoplasia type 1 (MEN1) organ manifestations and the increase in clinical experience warrant a... (Review)
Review
Multiple Endocrine Neoplasia Type 1 and the Pancreas: Diagnosis and Treatment of Functioning and Non-Functioning Pancreatic and Duodenal Neuroendocrine Neoplasia within the MEN1 Syndrome - An International Consensus Statement.
The better understanding of the biological behavior of multiple endocrine neoplasia type 1 (MEN1) organ manifestations and the increase in clinical experience warrant a revision of previously published guidelines. Duodenopancreatic neuroendocrine neoplasias (DP-NENs) are still the second most common manifestation in MEN1 and, besides NENs of the thymus, remain a leading cause of death. DP-NENs are thus of main interest in the effort to reevaluate recommendations for their diagnosis and treatment. Especially over the last 2 years, more clinical experience has documented the follow-up of treated and untreated (natural-course) DP-NENs. It was the aim of the international consortium of experts in endocrinology, genetics, radiology, surgery, gastroenterology, and oncology to systematically review the literature and to present a consensus statement based on the highest levels of evidence. Reviewing the literature published over the past decade, the focus was on the diagnosis of F- and NF-DP-NENs within the MEN1 syndrome in an effort to further standardize and improve treatment and follow-up, as well as to establish a "logbook" for the diagnosis and treatment of DP-NENs. This shall help further reduce complications and improve long-term treatment results in these rare tumors. The following international consensus statement builds upon the previously published guidelines of 2001 and 2012 and attempts to supplement the recommendations issued by various national and international societies.
Topics: Consensus; Duodenal Neoplasms; Humans; Multiple Endocrine Neoplasia Type 1; Pancreatic Neoplasms
PubMed: 32971521
DOI: 10.1159/000511791 -
Current Oncology (Toronto, Ont.) Sep 2022An increasing number of duodenal tumors are being diagnosed over the years, leading to increased confusion regarding the choice of treatment options. Small-to-large... (Review)
Review
An increasing number of duodenal tumors are being diagnosed over the years, leading to increased confusion regarding the choice of treatment options. Small-to-large tumors and histological types vary from adenoma to carcinoma, and treatment methods may need to be selected according to lesion characteristics. Because of its anatomic characteristics, complications are more likely to occur in the duodenum than in other gastrointestinal organs. Several reports have described the outcomes of conventional endoscopic mucosal resection, endoscopic submucosal dissection, cold snare polypectomy, underwater endoscopic mucosal resection, endoscopic full-thickness resection, and laparoscopic and endoscopic cooperative surgery for duodenal tumors. However, even in the guidelines set out by various countries, only the treatment methods are listed, and no clear treatment strategies are provided. Although there are few reports with a sufficiently high level of evidence, considering the currently available treatment options is essential. In this report, we reviewed previous reports on each treatment strategy, discussed the current issues and prospects, and proposed the best possible treatment strategy.
Topics: Humans; Duodenal Neoplasms; Colonic Polyps; Treatment Outcome; Colonoscopy; Duodenum; Adenoma
PubMed: 36290814
DOI: 10.3390/curroncol29100537 -
The Korean Journal of Gastroenterology... Apr 2021Superficial nonampullary duodenal epithelial tumors are considered rare but have been increasingly recognized in recent years. Accordingly, the importance of endoscopic... (Review)
Review
Superficial nonampullary duodenal epithelial tumors are considered rare but have been increasingly recognized in recent years. Accordingly, the importance of endoscopic treatment for the lesions are also increasing. An endoscopic resection can be considered for duodenal adenoma and mucosal cancer. The choice of resection method should be made based on the size of the lesion, endoscopic findings, pathologic diagnosis, and risk of procedure-related complication. For small adenomas <10 mm in size, endoscopic mucosal resection (EMR), cold snare polypectomy, and underwater EMR can be considered. An or piecemeal resection using EMR or underwater EMR can be selected for 10-20 mm sized adenomas. For lesions ≥20 mm in size or suspicious for mucosal cancer, an endoscopic submucosal dissection followed by closure of the mucosal defect conducted by an experienced endoscopist is appropriate.
Topics: Adenoma; Colonic Polyps; Colonoscopy; Duodenal Neoplasms; Endoscopic Mucosal Resection; Humans; Retrospective Studies; Treatment Outcome
PubMed: 33896904
DOI: 10.4166/kjg.2021.039 -
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 -
Archives of Pathology & Laboratory... Apr 2018Duodenal-type follicular lymphoma (D-FL) is a newly recognized entity in the 2016 World Health Organization classification update. It has an immunophenotype similar to... (Review)
Review
Duodenal-type follicular lymphoma (D-FL) is a newly recognized entity in the 2016 World Health Organization classification update. It has an immunophenotype similar to that of other FLs and usually carries the typical t(14;18)(q32;q21) translocation. However, unlike other FLs, D-FL is almost always diagnosed at a low stage and stays localized to the small intestine, most commonly the second portion of the duodenum, whereas the vast majority of other FLs are diagnosed at an advanced stage. Additionally, D-FL gene expression and pathogenesis appear to be more closely related to extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue than to other types of FL. Therefore, many oncologists have opted to treat this variant of FL in a "watch and wait" manner because of its excellent prognosis and the rarity of D-FL to progress even when no treatments are attempted.
Topics: Duodenal Neoplasms; Humans; Lymphoma, Follicular
PubMed: 29565210
DOI: 10.5858/arpa.2016-0519-RS -
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 -
The Journal of Histochemistry and... Nov 2021Non-ampullary duodenal adenocarcinoma (DAC) is a rare malignancy. Little information is available concerning the histopathological prognostic factors associated with...
Non-ampullary duodenal adenocarcinoma (DAC) is a rare malignancy. Little information is available concerning the histopathological prognostic factors associated with DAC. Carbonic anhydrases (CAs) are metalloenzymes catalyzing the universal reaction of CO hydration. Isozymes CAII, CAIX, and CAXII are associated with prognosis in various cancers. Our aim was to analyze the immunohistochemical expressions of CAII, CAIX, and CAXII in normal duodenal epithelium, duodenal adenomas, and adenocarcinoma and their associations with clinicopathological variables and survival. Our retrospective study included all 27 DACs treated in Oulu University Hospital during years 2000-2020. For comparison, samples of 42 non-ampullary adenomas were collected. CAII expression was low in duodenal adenomas and adenocarcinoma. CAIX expression in adenomas and adenocarcinoma was comparable with the high expression of normal duodenal crypts. Expression patterns in carcinomas were largely not related to clinicopathological features. However, low expression of CAII associated with poorer differentiation of the tumor (=0.049) and low expression of CAIX showed a trend for association with nodal spread, although statistical significance was not reached (=0.091). CAII and CAIX lost their epithelial polarization and staining intensity in adenomas. CAXII expression was not detected in the studied samples. CAs were not associated with survival. The prognostic value of CAII and CAIX downregulation should be further investigated. Both isozymes may serve as biomarkers of epithelial dysplasia in the duodenum.
Topics: Adenocarcinoma; Adult; Aged; Antigens, Neoplasm; Carbonic Anhydrase II; Carbonic Anhydrase IX; Cell Differentiation; Cohort Studies; Duodenal Neoplasms; Female; Humans; Male; Middle Aged
PubMed: 34636283
DOI: 10.1369/00221554211050133 -
Digestive Surgery 2021The management of the pancreas in patients with duodenal trauma or duodenal tumors remains a controversial issue. Pancreas-preserving total duodenectomy (PPTD) requires...
BACKGROUND
The management of the pancreas in patients with duodenal trauma or duodenal tumors remains a controversial issue. Pancreas-preserving total duodenectomy (PPTD) requires a meticulous surgical technique. The most common indication is familial duodenal adenomatous polyposis (FAP). The aims of this study are to carry out a systematic review of the literature on the indications for PPTD and to highlight the risks and benefits compared with other more aggressive procedures.
SUMMARY
A systematic literature review was performed following PRISMA recommendations of studies published in PubMed, Embase, and Cochrane library until May 2019. Thirty articles describing 211 patients were chosen. The mean age was 48 years. The surgical indication in 75% of patients was FAP. The mean operating time was 329 min and mean intraoperative bleeding 412 mL. Postoperative morbidity rate was 49.7% (76% Clavien-Dindo
97.8%. Key Messages: PPTD is indicated for patients with benign and premalignant duodenal lesions without involvement of the pancreatic head. It is a feasible procedure offering an alternative to other more aggressive procedures in selected patients. Mortality is below 1.5%. Topics: Adenomatous Polyposis Coli; Digestive System Surgical Procedures; Duodenal Neoplasms; Duodenum; Humans; Pancreas; Postoperative Complications
PubMed: 34000717
DOI: 10.1159/000515718