scholarly journals S1428 Occurrence of Gastric Cancer in Patients with Juvenile Polyposis Syndrome: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 116 (1) ◽  
pp. S655-S655
Author(s):  
Achintya D. Singh ◽  
Akshita Gupta ◽  
Amit Bhatt ◽  
Carol A. Burke
2019 ◽  
Vol 57 (04) ◽  
pp. 497-500
Author(s):  
Moritz Schiemer ◽  
Annette Schmitt-Graeff ◽  
Volker Brass ◽  
Peter Hasselblatt

AbstractJuvenile polyposis syndrome is a rare autosomal-dominant disorder characterized by multiple hamartomatous polyps in the gastrointestinal tract. It is associated with an increased risk of gastrointestinal cancer. We report the case of a 49-year-old woman presenting with proximal muscle weakness, weight loss, severe anemia, and melena. One year before, the diagnosis of a “fundic gland polyposis” was presumed after endoscopic evaluation for iron deficiency anemia had shown numerous polyps limited to the gastric mucosa. On admission, the diagnosis of dermatomyositis was made based on laboratory results with a marked elevated creatine kinase as well as the presence of characteristic clinical findings and muscle histology. Upper endoscopy revealed multiple pedunculated, edematous polyps in the stomach without apparent cancerous lesions intraluminally. Infiltration of the muscular layer was not detectable on endoscopic ultrasound. Histopathological examination of the polyps showed smooth outer surfaces and multiple dilated cystic glands, consistent with hamartomatous juvenile-type polyps. Magnetic resonance imaging revealed a peritoneal mass close to the greater curvature of the stomach, which was identified as a poorly differentiated adenocarcinoma by laparoscopic sampling. Immunohistochemical analysis of resected polyps was remarkable for a loss of SMAD4 expression, a finding that is very commonly observed in patients with gastric juvenile polyposis syndrome. Despite initial treatment response to glucocorticoids and chemotherapy, the patient died 5 months later due to progressive illness. Patients with gastric juvenile polyposis and SMAD4 mutations are at a high risk of developing gastric cancer; hence, early gastrectomy should be considered.


2012 ◽  
Vol 30 (25) ◽  
pp. e229-e232 ◽  
Author(s):  
Zsofia K. Stadler ◽  
Erin Salo-Mullen ◽  
Liying Zhang ◽  
Jinru Shia ◽  
Ruben Bacares ◽  
...  

2018 ◽  
Author(s):  
Ji Cheng ◽  
Ming Cai ◽  
Xiaoming Shuai ◽  
Jinbo Gao ◽  
Guobin Wang ◽  
...  

2020 ◽  
Vol 13 (12) ◽  
pp. e236855
Author(s):  
Wendy Chang ◽  
Patricia Renaut ◽  
Casper Pretorius

Juvenile polyposis syndrome (JPS) and hereditary haemorrhagic telangiectasia (HHT) are rare autosomal dominant diseases, where symptoms manifest at childhood. A 32-year-old man with no family history of JPS or HHT with SMAD4 gene mutation who developed signs and symptoms only at the age of 32, when he was an adult. In this article, we highlight the steps taken to diagnose this rare pathology, explain its pathophysiology and management.


BMJ Open ◽  
2020 ◽  
Vol 10 (1) ◽  
pp. e033267
Author(s):  
Dengfeng Wang ◽  
Yang Yu ◽  
Pengxian Tao ◽  
Dan Wang ◽  
Yajing Chen ◽  
...  

IntroductionVenous thromboembolism (VTE) is a serious life-threatening complication in patients with gastric cancer. Abnormal coagulation function and tumour-related treatment may contribute to the occurrence of VTE. Many guidelines considered that surgical treatment would put patients with cancer at high risk of VTE, so positive prevention is needed. However, there are no studies that have systematically reviewed the postoperative risk and distribution of VTE in patients with gastric cancer. We thus conduct this systematic review to determine the risk of VTE in patients with gastric cancer undergoing surgery and provide some evidence for clinical decision-making.Methods and analysisStudies reporting the incidence of VTE after gastric cancer surgery will be included. Primary studies of randomised controlled trials, cohort studies, population-based surveys and cross-sectional studies are eligible for this review and only studies published in Chinese and English will be included. We will search the Medline, Embase, Web of Science, CBM, CNKI and Wanfang data from their inception to November 2019. Two reviewers will independently select studies and extract data. The quality of each included study will be assessed with tools corresponding to their study design. Meta-analysis will be used to pool the incidence data from included studies. Heterogeneity of the estimates across studies will be assessed, if necessary, a subgroup analysis will be performed to explore the source of heterogeneity. The Grades of Recommendation, Assessment, Development and Evaluation method is applied to assess the level of evidence obtained from this systematic review.Ethics and disseminationThis proposed systematic review and meta-analysis is based on published data, and thus ethical approval is not required. The results of this review will be sought for publication.PROSPERO registration numberCRD42019144562


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