Role of endoscopic ultrasound in the characterization of solid pseudopapillary neoplasm of the pancreas

BACKGROUND Solid pseudopapillary neoplasm(SPN)is an uncommon pathology of the pancreas with unpredictable malignant potential.Endoscopic ultrasound(EUS)assessment plays a vital role in lesion characterization and confirmation of the tissue diagnosis.However,there is a paucity of data regarding the imaging assessment of these lesions.AIM To determine the characteristic EUS features of SPN and define its role in preoperative assessment.METHODS This was an international,multicenter,retrospective,observational study of prospective cohorts from 7 large hepatopancreaticobiliary centers.All cases with postoperative histology of SPN were included in the study.Data collected included clinical,biochemical,histological and EUS characteristics.RESULTS One hundred and six patients with the diagnosis of SPN were included.The mean age was 26 years(range 9 to 70 years),with female predominance(89.6%).The most frequent clinical presentation was abdominal pain(80/106;75.5%).The mean diameter of the lesion was 53.7 mm(range 15 to 130 mm),with the slight predominant location in the head of the pancreas(44/106;41.5%).The majority of lesions presented with solid imaging features(59/106;55.7%)although 33.0%(35/106)had mixed solid/cystic characteristics and 11.3%(12/106)had cystic morphology.Calcification was observed in only 4(3.8%)cases.Main pancreatic duct dilation was uncommon,evident in only 2 cases(1.9%),whilst common bile duct dilation was observed in 5(11.3%)cases.One patient demonstrated a double duct sign at presentation.Elastography and Doppler evaluation demonstrated inconsistent appearances with no emergence of a predictable pattern.EUS guided biopsy was performed using three different types of needles:Fine needle aspiration(67/106;63.2%),fine needle biopsy(37/106;34.9%),and Sonar Trucut(2/106;1.9%).The diagnosis was conclusive in 103(97.2%)cases.Ninety-seven patients were treated surgically(91.5%)and the post-surgical SPN diagnosis was confirmed in all cases.During the 2-year follow-up period,no recurrence was observed.CONCLUSION SPN presented primarily as a solid lesion on endosonographic assessment.The lesion tended to be located in the head or body of the pancreas.There was no consistent characteristic pattern apparent on either elastography or Doppler assessment.Similarly SPN did not frequently cause stricture of the pancreatic duct or common bile duct.Importantly,we confirmed that EUS-guided biopsy was an efficient and safe diagnostic tool.The needle type used does not appear to have a significant impact on the diagnostic yield.Overall SPN remains a challenging diagnosis based on EUS imaging with no pathognomonic features.EUS guided biopsy remains the gold standard in establishing the diagnosis.

Clinical characteristics and outcomes in carbohydrate antigen 19-9 negative pancreatic cancer

BACKGROUND Pancreatic ductal adenocarcinoma(PDAC)is a leading cause of death from cancer worldwide.Tumor markers like carbohydrate antigen 19-9(CA 19-9)have been proven valuable as a diagnostic tool and a predictor for tumor staging and response to therapy.AIM To delineate the phenotype of normal CA 19-9 PDAC according to clinical features,disease staging and prognosis as compared with high CA 19-9 PDAC cases.METHODS We performed a retrospective single-center analysis of all PDAC cases admitted in our Gastroenterology department over a period of 30 mo that were diagnosed by endoscopic ultrasound-guided tissue acquisition.Patients were divided into two groups according to CA 19-9 levels over a threshold of 37 U/mL.We performed a comparison between the two groups with regard to demographic and clinical data,biomarkers,tumor staging and 6-mo survival.RESULTS Altogether 111 patients were recruited with 29 having documented normal CA 19-9(<37 U/mL).In the CA 19-9 negative group of patients,20.68%had elevated levels of both CEA and CA 125,13.79%for CA 125 only whilst 17.24%for CEA only.The two groups had similar demographic characteristics.Abdominal pain was more frequently reported in positive vs negative CA 19-9 PDAC cases(76.83%vs 55.17%),while smoking was slightly more prevalent in the latter group(28.04%vs 31.03%).Tumors over 2 cm were more frequently seen in the positive CA 19-9 group,reflecting a higher proportion of locally advanced and metastatic neoplasia(87.7%vs 79.3%).Sixmonth survival was higher for the negative CA 19-9 group(58.62%vs 47.56%).CONCLUSION Elevated CA 19-9 at diagnosis seems to be associated with a more pronounced symptomatology,high tumor burden and poor prognosis compared to negative CA 19-9 PDAC cases.CEA and CA 125 can be adjunctive useful markers for PDAC,especially in CA 19-9 negative cases.

Pancreatic involvement in celiac disease

Celiac disease(CD)is well recognized as a systemic,chronic autoimmune disease mainly characterized by gluten-sensitive enteropathy in genetically predisposed individuals but with various extraintestinal features.One of the affected organs in CD is the pancreas,consisting of both endocrine and exocrine alterations.Over the last decades there has been increasing interest in the pancreatic changes in CD,and this has been reflected by a great number of publications looking at this extraintestinal involvement during the course of CD.While pancreatic endocrine changes in CD,focusing on type 1 diabetes mellitus,are well documented in the literature,the relationship with the exocrine pancreas has been less studied.This review summarizes currently available evidence with regard to pancreatic exocrine alterations in CD,focusing on risk of pancreatitis in CD patients,association with autoimmune pancreatitis,prevalence and outcomes of pancreatic exocrine insufficiency in newly diagnosed and gluten-free diet treated CD patients,and the link with cystic fibrosis.In addition,we discuss mechanisms behind the associated pancreatic exocrine impairment in CD and highlight the recommendations for clinical practice.

Celiac crisis, a rare occurrence in adult celiac disease: A systematic review

BACKGROUND Celiac crisis(CC), a potentially life-threatening condition, is one of the rare clinical presentations of celiac disease(CD). Several cases have been documented in the literature, mostly in children.AIM To perform a review of CC cases reported in adult CD patients.METHODS A systematic search of the literature was conducted in two databases,PubMed/MEDLINE and EMBASE, using the term "celiac crisis" and its variant"coeliac crisis", from January 1970 onwards. Altogether, 29 articles reporting 42 biopsy-proven cases were found in the search. Here, we summarized the demographic, clinical characteristics, laboratory and diagnostic work-ups, and therapeutic management in these patients.RESULTS Among the 42 CD cases, the median age was 50 years(range 23-83), with a 2:1 female to male ratio. The majority of patients(88.1%) developed CC prior to CDdiagnosis, while the remaining were previously diagnosed CD cases reporting low adherence to a gluten-free diet(GFD). Clinically, patients presented with severe diarrhea(all cases), weight loss(about two thirds) and, in particular situations, with neurologic(6 cases) or cardiovascular(1 case) manifestations or bleeding diathesis(4 cases). One in four patients had a precipitating factor that could have triggered the CC(e.g. trauma, surgery, infections). Laboratory workup of patients revealed a severe malabsorptive state with metabolic acidosis,dehydration, hypoalbuminemia and anemia. The evolution of GFD was favorable in all cases except one, in whom death was reported due to refeeding syndrome.CONCLUSION Celiac crisis is a rare but severe and potentially fatal clinical feature of CD. A high index of suspicion is needed to recognize this clinical entity and to deliver proper therapy consisting of supportive care and, subsequently, GFD.

Celiac disease and Sjögren’s syndrome:A case report and review of literature

BACKGROUND Celiac disease(CD)is a systemic,chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals,with a prevalence of 1%worldwide.Sjogren's syndrome(SS)is also a systemic autoimmune disease,mainly characterized by ocular and oral sicca symptoms and signs.Sharing a common genetic background,CD and SS are known associated autoimmune diseases,but currently available guidelines are not reporting it.CASE SUMMARY We report the case of a 39-year-old woman,who was in the care of her rheumatologist for 2 years with SS.On routine follow-up she was found to have iron deficiency,without anemia.She had no gastrointestinal complaints and denied any obvious source of blood loss.IgA tissue transglutaminase antibodies were positive and endoscopy with duodenal biopsies revealed crypt hyperplasia and villous atrophy.A diagnosis of CD was set and gluten-free diet was recommended.CONCLUSION We present a review of existing data in the literature regarding the association of the two diseases,summarizing prevalence studies of CD in SS patients and the other way around.Screening recommendations and future research perspectives are also discussed,highlighting clinically relevant unanswered questions with respect to the association of CD with SS.

Abdominal imaging in COVID-19

Initially thought of as a respiratory infection,coronavirus disease-2019(COVID-19)is now recognized as a complex disease with a wide clinical spectrum,including digestive involvement.While several studies have evaluated chest imaging findings in COVID-19,few papers have looked at the abdominal imaging features of these patients.Liver,biliary,pancreas and bowel involvement have been reported in COVID-19 infected patients.In this review,we aim to summarize currently available data related to abdominal imaging techniques in COVID-19,in accordance with relevant clinical and laboratory workup of these patients.Underlying mechanisms,indications and imaging findings related to COVID-19 are discussed based on published data.Also,practice points for clinicians are highlighted in order to adequately recognize digestive-related injuries of severe acute respiratory syndrome coronavirus 2 infection.While there’s been a steady accumulation of data with respect to abdominal imaging findings in COVID-19,currently available recommendations are based on limited research.There is a wide spectrum of abdominal imaging findings in COVID-19,which includes hepato-biliary,pancreatic and luminal pathology.

Digital histology in celiac disease:A practice changer

Artificial intelligence(AI)has grown tremendously in the last decades and is undoubtedly the future era in medicine.Concerning digestive diseases,applications of AI include clinical gastroenterology,gastrointestinal endoscopy and imaging,and not least pathological diagnosis.Several gastrointestinal pathologies require histological confirmation for a positive diagnosis.Among them,celiac disease(CD)diagnosis has been in the spotlight over time,but controversy is still ongoing with regard to the so-called celiac-type histology.Despite efforts to improve histological diagnosis in CD,there are still several issues and pitfalls associated with duodenal histology reading.Several papers have assessed the accuracy of AI techniques in detecting CD on duodenal biopsy images and have shown high diagnostic performance over standard histology reading.We discuss the role of computer-assisted histology in improving the assessment of mucosal architectural injury and inflammation in CD patients,both for diagnosis and follow-up.