[Hereditary and familial forms of pancreatic adenocarcinoma: Genetic determinism, patients eligible for systematic screening, screening methods and results]. / Les formes héréditaires et familiales des adénocarcinomes pancréatiques : déterminisme génétique, population éligible à un dépistage systématique, modalités et résultats du dépistage.

Systematic screening for pancreatic cancer in high risk individuals is justified by the poor prognosis of the majority of cases diagnosed at a symptomatic stage that are mostly advanced and unresectable Individual risk assessment is based on both genetic data and family history. The screening of a panel of susceptibiility genes should be offered to any affected individual when a genetic predisposition is suspected. An international consortium has proposed a definition of the at risk population, candidate for screening, and there is a consensus on the target lesions of this screening: early adenocarcinoma and benign lesions with a high potential for malignant transformation: Intraductal Papillary Mucinous Neopasm (IPMN) and Pancreatic Intraepithelial Neoplasia (PanIN) with high-grade dysplasia. Its modalities currently consist of an annual pancreatic MRI and/or endoscopic ultrasound (EUS), associated with screening for diabetes mellitus. The main limitation of screening, the effectiveness of which has not yet been demonstrated, is its lack of sensitivity, which results in a non-negligible rate of interval cancers and sometimes advanced diagnoses. Insufficient specificity is also imperfect, in particular with regard to benign lesions with a low potential for degeneration, and can lead to the proposal of unjustified surgeries. This situation makes the future integration of new imaging techniques and promising new biological approaches that are being explored highly desirable.

Endoscopic submucosal dissection versus endoscopic mucosal resection for early esophageal adenocarcinoma.

OBJECTIVES: Endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD) allow endoscopic resection of early esophageal adenocarcinoma. The choice between the two techniques takes into account the morphology of the lesion, and the experience of the endoscopist. The aim of this study was to compare EMR to ESD for the treatment of early esophageal adenocarcinoma. METHODS: Patients who underwent an endoscopic resection for esophageal adenocarcinomas between March 2015 and December 2019 were included. ESD was compared to EMR in terms of clinical, procedural, histologic, and oncologic outcomes. RESULTS: 85 patients were included: 57 ESD and 28 EMR. The median (IQR) diameter of the lesion was 20(15-25) mm in the ESD group, and 15(8-16) mm in the EMR group, p<0.01. ESD allowed en bloc resection in 100% of cases, and EMR in 39% of cases, p<0.001. The R0 and curative resection rate in the ESD group versus the EMR group were 88% and 67%, respectively, versus 21% and 11%, p<0.001. We recorded one severe adverse event, in the EMR group. After a median (IQR) follow-up of 27.5 (14.5-38.7) months, the local recurrence rate was 23% vs. 18% (p = 0.63), and the overall survival 89% vs. 86% (p = 0.72), in the ESD and EMR groups, respectively. CONCLUSION: ESD was as safe as EMR and allowed higher en bloc, R0 and curative resection rates. Although these results did not translate into long-term outcomes, these data prompt for a broader adoption of ESD for the resection of esophageal lesions suspected of harboring early esophageal adenocarcinoma.

Efficacy of Organ Preservation Strategy by Adjuvant Chemoradiotherapy after Non-Curative Endoscopic Resection for Superficial SCC: A Multicenter Western Study.

BACKGROUND: In case of high risk of lymph node invasion after endoscopic resection (ER) of superficial esophageal squamous cell carcinoma (SCC), adjuvant chemoradiotherapy (CRT) can be an alternative to surgery. We assessed long-term clinical outcomes of adjuvant therapy by CRT after non-curative ER for superficial SCC. METHODS: We performed a retrospective multicenter study. From April 1999 to April 2018, all consecutive patients who underwent ER for SCC with tumor infiltration beyond the muscularis mucosae were included. RESULTS: A total of 137 ER were analyzed. The overall nodal or metastatic recurrence-free survival rate at 5 years was 88% and specific recurrence-free survival rates at 5 years with and without adjuvant therapy were, respectively, 97.9% and 79.1% (p = 0.011). Independent factors for nodal and/or distal metastatic recurrence were age (HR = 1.075, p = 0.031), Sm infiltration depth > 200 µm (HR = 4.129, p = 0.040), and the absence of adjuvant CRT or surgery (HR = 11.322, p = 0.029). CONCLUSION: In this study, adjuvant therapy is associated with a higher recurrence-free survival rate at 5 years after non-curative ER. This result suggests this approach may be considered as an alternative to surgery in selected patients.

Outcomes of rescue procedures in the management of locally recurrent ampullary tumors: A Pancreas 2000/EPC study.

BACKGROUND: Ampullary lesions are rare and can be locally treated either with endoscopic papillectomy or transduodenal surgical ampullectomy. Management of local recurrence after a first-line treatment has been poorly studied. METHODS: Patients with a local recurrence of an ampullary lesion initially treated with endoscopic papillectomy or transduodenal surgical ampullectomy were retrospectively included from a multi-institutional database (58 centers) between 2005 and 2018. RESULTS: A total of 103 patients were included, 21 (20.4%) treated with redo endoscopic papillectomy, 14 (13.6%) with transduodenal surgical ampullectomy, and 68 (66%) with pancreaticoduodenectomy. Redo endoscopic papillectomy had low morbidity with 4.8% (n = 1) severe to fatal complications and a R0 rate of 81% (n = 17). Transduodenal surgical ampullectomy and pancreaticoduodenectomy after a first procedure had a higher morbidity with Clavien III and more complications, respectively, 28.6% (n = 4) and 25% (n = 17); R0 resection rates were 85.7% (n = 12) and 92.6% (n = 63), both without statistically significant difference compared to endoscopic papillectomy (P = .1 and 0.2). Pancreaticoduodenectomy had 4.4% (n = 2) mortality. No deaths were registered after transduodenal surgical ampullectomy or endoscopic papillectomy. Recurrences treated with pancreaticoduodenectomy were more likely to be adenocarcinomas (79.4%, n = 54 vs 21.4%, n = 3 for transduodenal surgical ampullectomy and 4.8%, n = 1 for endoscopic papillectomy, P < .0001). Three-year overall survival and disease-free survival were comparable. CONCLUSION: Endoscopy is appropriate for noninvasive recurrences, with resection rate and survival outcomes comparable to surgery. Surgery applies more to invasive recurrences, with transduodenal surgical ampullectomy rather for carcinoma in situ and early cancers and pancreaticoduodenectomy for more advanced tumors.

Outcomes of endoscopic mucosal resection for large superficial non-ampullary duodenal adenomas.

Endoscopic mucosal resection (EMR) is the recommended treatment for superficial non-ampullary duodenal epithelial tumors larger than 6 mm. This endoscopic technique carries a high risk of adverse events. Our aim was to identify the risk factors for adverse events following EMR for non-ampullary duodenal adenomatous lesions. We retrospectively analyzed a prospectively collected database of consecutive endoscopic resections for duodenal lesions at a tertiary referral center for therapeutic endoscopy. We analyzed patients with non-ampullary duodenal adenomatous lesions ≥ 10 mm resected by EMR, and searched for factors associated with adverse events after EMR. 167 duodenal adenomatous lesions, with a median size of 25 (25-40) mm, were resected by EMR between January 2015 and December 2020. Adverse events occurred in 37/167 (22.2%) after endoscopic resection, with 29/167 (17.4%) delayed bleeding, 4/167 (2.4%) immediate perforation and 4/167 (2.4%) delayed perforation. In logistic regression, the size of the lesion was the only associated risk factor of adverse events (OR = 2.81, 95% CI [1.27; 6.47], p = 0.012). Adverse events increased mean hospitalization time (7.7 ± 9 vs. 1.9 ± 1 days, p < 0.01). None of the currently recommended preventive methods, particularly clips, affected the adverse event rate. EMR of centimetric and supracentimetric duodenal adenomatous lesions carries a high risk of adverse events, increasing with the size of the lesion and with no benefit from any preventive method. These results suggest that these procedures should be performed in expert centers, and underline the need for novel endoscopic tools to limit the rate of adverse events.

Endoscopic ultrasound guided fine needle biopsy in patients with suspected gastric linitis plastica.

BACKGROUND: Gastric linitis plastica (GLP) is a diffuse infiltrating type of gastric adenocarcinoma. It is associated with a poor prognosis and a five-year survival of 3-10%. The infiltrating profile of this tumor explains the low yield of the superficial mucosal biospies. The objective of this study was to investigate the role of endoscopic ultrasound-fine needle biopsy (EUS-FNB) in the diagnosis of GLP. METHODS: We performed a retrospective analysis including all patients who had an EUS-FNB, at a tertiary referral center, over the last 3 years. The primary outcome was the sensitivity of EUS-FNB in patients with suspected GLP. RESULTS: Between January 2017 and December 2020, 34 patients had an EUS-FNB for suspected GLP. Ten patients had a diagnostic of GLP. This diagnosis was obtained by EUS-FNB in 90% (9/10) of the cases. Eight patients had at least one previous esophagogastroduodenoscopy (EGD) with negative mucosal biopsies. Gastric EUS-FNB helped diagnose other serious conditions in 47% (16/34) of cases with inconclusive mucosal biopsies. CONCLUSION: Gastric EUS-FNB in patients with suspected GLP and normal endoscopic mucosal biopsies may lead to a positive diagnosis of GLP in 90% of cases without notable adverse events. This technique should be considered as a second step in the setting of suspicion of GLP after inconclusive mucosal biopsies.

Endoscopic management of non-ampullary duodenal adenomas.

Duodenal polyps are found in 0.1 % to 0.8 % of all upper endoscopies. Duodenal adenomas account for 10 % to 20 % of these lesions. They can be sporadic or occur in the setting of a hereditary predisposition syndrome, mainly familial adenomatous polyposis. Endoscopy is the cornerstone of management of duodenal adenomas, allowing for diagnosis and treatment, primarily by endoscopic mucosal resection. The endoscopic treatment of duodenal adenomas has a high morbidity, reaching 15 % in a prospective study, consisting of bleeding and perforations, and should therefore be performed in expert centers. The local recurrence rate ranges from 9 % to 37 %, and is maximal for piecemeal resections of lesions > 20 mm. Surgical resection of the duodenum is flawed with major morbidity and considered a rescue procedure in cases of endoscopic treatment failures or severe endoscopic complications such as duodenal perforations. In this paper, we review the existing evidence on endoscopic diagnosis and treatment of non-ampullary duodenal adenomas.

Amplatzer occluders for refractory esophago-respiratory fistulas: a case series.

Background and study aims Endoscopic management of esophagorespiratory fistulas (ERF) is challenging and currently available options (stents, double pigtail, endoscopic vacuum therapy) are not very effective. We report the feasibility and efficacy of endoscopic placement of Amplatzer cardiovascular occluders for this indication. Patients and methods This was a single-center, prospective study (June 2019 to September 2020) of all patients with non-malignant ERF persistent after conventional management with esophageal and/or tracheal stents. The primary outcome was the technical feasibility of Amplatzer placement. Secondary outcomes were clinical success defined by effective ERF occlusion and resolution of respiratory symptoms allowing oral food intake. Results Endoscopic placement of Amplatzer occluders was feasible in 83 % of patients (5/6), with a 50 % (3/6) clinical success rate at 9 months. The mortality rate was 33 % (2/6). Conclusions An Amplatzer cardiac or vascular occluder is a feasible and safe treatment option for refractory ERF, with a 50 % short-term clinical success.

Air circulation in a gastrointestinal light source box and endoscope in the era of SARS-CoV-2 and airborne transmission of microorganisms.

Background and study aims The role that air circulation through a gastrointestinal endoscopy system plays in airborne transmission of microorganisms has never been investigated. The aim of this study was to explore the potential risk of transmission and potential improvements in the system. Methods We investigated and described air circulation into gastrointestinal endoscopes from Fujifilm, Olympus, and Pentax. Results The light source box contains a lamp, either Xenon or LED. The temperature of the light is high and is regulated by a forced-air cooling system to maintain a stable temperature in the middle of the box. The air used by the forced-air cooling system is sucked from the closed environment of the patient through an aeration port, located close to the light source and evacuated out of the box by one or two ventilators. No filter exists to avoid dispersion of particles outside the processor box. The light source box also contains an insufflation air pump. The air is sucked from the light source box through one or two holes in the air pump and pushed from the air pump into the air pipe of the endoscope through a plastic tube. Because the air pump does not have a dedicated HEPA filter, transmission of microorganisms cannot be excluded. Conclusions Changes are necessary to prevent airborne transmission. Exclusive use of an external CO 2 pump and wrapping the endoscope platform with a plastic film will limit scatter of microorganisms. In the era of pandemic virus with airborne transmission, improvements in gastrointestinal ventilation systems are necessary to avoid contamination of patients and health care workers.

Study Protocol of the ESAP Study: Endoscopic Papillectomy vs. Surgical Ampullectomy vs. Pancreaticoduodenectomy for Ampullary Neoplasm-A Pancreas2000/EPC Study.

Background: Lesions of the Ampulla of Vater are a rare condition and represent <10% of peri-ampullary neoplasms. Nevertheless, ampullary adenomas have the potential for malignant transformation to ampullary carcinomas by an adenoma-to-carcinoma sequence. Thus, adequate patient selection and complete resection (R0) of non-invasive ampullary lesions either by endoscopic papillectomy (EP), surgical ampullectomy (SA), or pancreaticoduodenectomy (PD) is essential. Although PD was traditionally performed, recent studies reported considerable efficacy and fewer complications following EP and SA. Since consistent comparative data are lacking, the Endoscopic Papillectomy vs. Surgical Ampullectomy vs. Pancreaticoduodectomy (ESAP) study will provide evidence for a therapeutic standard and post procedure morbidity in ampullary lesions. Methods: International multicenter retrospective study. Adult patients (>18 years of age) who underwent SA or PD for ampullary neoplasm between 2004 and 2018 or EP between 2007 and 2018 will be evaluated. Main inclusion criteria are ampullary lesions strictly located to the ampulla. This includes adenoma, adenocarcinoma (T1 and T2), neuroendocrine tumors, gastrointestinal stroma tumors and other rare conditions. Exclusion criteria are peri-ampullary lesions, e.g., from the duodenal wall or the head of the pancreas, and interventions for tumor stages higher than T2. The main objective of this study is to analyze rates of complete resection (R0), recurrence and necessity for complementary interventions following EP, SA, and PD. Treatment-quality for each procedure will be defined by morbidity, mortality and complication rates and will be compared between EP, SA, and PD. Secondary objectives include outcome for patients with incomplete resection or initially understated tumors, lesions of the minor papilla, hereditary syndromes, neuroendocrine tumors, mesenchymal lesions, and other rare conditions. Additionally, we will analyze therapy by argon plasma coagulation and radiofrequency ablation. Furthermore, outcome in curative and palliative interventions can be distinguished. Conclusion: The ESAP study will provide evidence for therapeutic algorithms and data for the implementation of guidelines in the treatment of different types of ampullary tumors, including recurrent, or incomplete resected lesions.

Development and validation of an automated algorithm to evaluate the abundance of bubbles in small bowel capsule endoscopy.

BACKGROUND AND STUDY AIMS: Bubbles can impair visualization of the small bowel (SB) mucosa during capsule endoscopy (CE). We aimed to develop and validate a computed algorithm that would allow evaluation of the abundance of bubbles in SB-CE still frames. PATIENTS AND METHODS: Two sets of 200 SB-CE normal still frames were created. Two experienced SB-CE readers analyzed both sets of images twice, in a random order. Each still frame was categorized as presenting with < 10 % or ≥ 10 % of bubbles. Reproducibility (κ), sensitivity (Se), specificity (Sp), receiver operating characteristic curve, and calculation time were measured for different algorithms (Grey-level of co-occurrence matrix [GLCM], fractal dimension, Hough transform, and speeded-up robust features [SURF]) using the experts' analysis as reference. Algorithms with highest reproducibility, Se and Sp were then selected for a validation step on the second set of frames. Criteria for validation were κ = 1, Se ≥ 90 %, Sp ≥ 85 %, and a calculation time < 1 second. RESULTS: Both SURF and GLCM algorithms had high operating points (Se and Sp over 90 %) and a perfect reproducibility (κ = 1). The validation step showed the GLCM detector strategy had the best diagnostic performances, with a Se of 95.79 %, a Sp of 95.19 %, and a calculation time of 0.037 seconds per frame. CONCLUSION: A computed algorithm based on a GLCM detector strategy had high diagnostic performance allowing assessment of the abundance of bubbles in SB-CE still frames. This algorithm could be of interest for clinical use (quality reporting) and for research purposes (objective comparison tool of different preparations).