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      Duodenal Gastrointestinal Stromal Tumor: A Rare Disease in a Young Adult Female Patient Presenting with Life-Threatening Hemorrhage

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          Abstract

          Duodenal gastrointestinal stromal tumors (dGISTs) may be a source of life-threatening hemorrhage that leads to emergency surgical care, precluding tumor staging and the planning of an elective treatment. In this study, we report a case of potentially lethal bleeding dGIST in a young woman successfully treated by an organ-preserving elective surgery after endoscopic and angiographic hemostasis. A 26-year-old female patient was admitted to the Emergency Unit of our hospital with the complaints of hematemesis and melena in the previous 12 h. An upper endoscopy showed a 4-cm submucosal lesion, between the 2nd and 3rd part of the duodenum, in the lateral wall, with massive bleeding arising from central ulceration. Hemostasis was initially achieved endoscopically and then optimized by transarterial embolization. After a contrast-enhanced CT, the patient underwent planning elective surgery. Intraoperatively, a 3-cm lesion was confirmed and resected by excision of the full-thickness duodenum with adequate free margins. Immunohistochemical analysis of the specimen revealed to be a dGIST, with a low mitotic count (<5 mitosis/50 high power field), and tumor necrosis present in <50% of the lesion. The patient had an uneventful course.

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          Most cited references15

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          Gastrointestinal stromal tumors: pathology and prognosis at different sites.

          Gastrointestinal (GI) stromal tumors (GISTs) are the most common mesenchymal tumors specific to the GI tract, generally defined as KIT (CD117)-positive tumors with a characteristic set of histologic features. These tumors, derived from Cajal cells or their precursors, most commonly occur at the age >50 years in the stomach (60%), jejunum and ileum (30%), duodenum (4-5%), rectum (4%), colon and appendix (1-2%), and esophagus ( 5 per 50 HPFs and >5 cm in diameter have a high risk for metastasis. In contrast, all intestinal GISTs >5 cm independent of mitotic rate have at least moderate risk for metastases, and all >5 mitoses per 50 HPFs have a high risk for metastases. Intestinal GISTs < or =5 cm with < or =5 mitoses per 50 HPFs have a low risk for metastases. Gastric GISTs can be divided into histologic subgroups including 4 spindle cell and 4 epithelioid variants. Intestinal GISTs are a histologically more homogeneous group and often contain distinctive extracellular collagen globules, skeinoid fibers. Immunohistochemical demonstration of KIT, CD34, or protein kinase theta positivity helps to properly identify these tumors.
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            Risk Associated With Complications and Mortality After Urgent Surgery vs Elective and Emergency Surgery: Implications for Defining "Quality" and Reporting Outcomes for Urgent Surgery.

            Given the current climate of outcomes-driven quality reporting, it is critical to appropriately risk stratify patients using standardized metrics.
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              Gastrointestinal stromal tumour.

              Gastrointestinal stromal tumours are the most common mesenchymal neoplasm of the gastrointestinal tract and are highly resistant to conventional chemotherapy and radiotherapy. Such tumours usually have activating mutations in either KIT (75-80%) or PDGFRA (5-10%), two closely related receptor tyrosine kinases. These mutations lead to ligand-independent activation and signal transduction mediated by constitutively activated KIT or PDGFRA. Targeting these activated proteins with imatinib mesylate, a small-molecule kinase inhibitor, has proven useful in the treatment of recurrent or metastatic gastrointestinal stromal tumours and is now being tested as an adjuvant or neoadjuvant. However, resistance to imatinib is a growing problem and other targeted therapeutics such as sunitinib are available. The important interplay between the molecular genetics of gastrontestinal stromal tumour and responses to targeted therapeutics serves as a model for the study of targeted therapies in other solid tumours.
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                Author and article information

                Journal
                Case Rep Gastroenterol
                Case Rep Gastroenterol
                CRG
                Case Reports in Gastroenterology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                1662-0631
                May-Aug 2021
                14 June 2021
                14 June 2021
                : 15
                : 2
                : 519-524
                Affiliations
                Fondazione IRCCS Cà Granda, Maggiore Policlinico Hospital, Milan, Italy
                Author notes
                Article
                crg-0015-0519
                10.1159/000515370
                8454229
                de09ecfb-6a69-485a-b165-9b2b8c58faa8
                Copyright © 2021 by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                : 20 December 2020
                : 17 February 2021
                : 2021
                Page count
                Figures: 4, References: 15, Pages: 6
                Categories
                Single Case

                Gastroenterology & Hepatology
                bleeding,duodenal gastrointestinal stromal tumors,life-threatening acute hemorrhage,young patient

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