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      GIST apendicular Translated title: Appendicular GIST

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          Abstract

          El tumor del estroma gastrointestinal (Gastrointestinal Stromal Tumor, GIST), localizado en el apéndice, constituye una entidad poco frecuente. Su cuadro clínico e imaginológico es indistinguible de una apendicitis aguda, por lo que su diagnóstico definitivo está dado por el estudio histopatológico e inmunohistoquímico. El tratamiento estándar es la apendicectomía. El propósito de este artículo es la presentación de un caso clínico y la revisión de la literatura.

          Translated abstract

          The gastrointestinal stromal tumor (GIST) located in the appendix is a rare entity. It is clinical indistinguishable from acute appendicitis, so definitive diagnosis is given by the histopathological and immunohistochemical studies. The standard treatment is appendectomy. The purpose of this article is presenting a case study and review of the literature.

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          Most cited references33

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          Biology of gastrointestinal stromal tumors.

          Once a poorly defined pathologic oddity, in recent years, gastrointestinal stromal tumor (GIST) has emerged as a distinct oncogenetic entity that is now center stage in clinical trials of kinase-targeted therapies. This review charts the rapid progress that has established GIST as a model for understanding the role of oncogenic kinase mutations in human tumorigenesis. Approximately 80% to 85% of GISTs harbor activating mutations of the KIT tyrosine kinase. In a series of 322 GISTs (including 140 previously published cases) studied by the authors in detail, mutations in the KIT gene occurred with decreasing frequency in exons 11 (66.1%), 9 (13%), 13 (1.2%), and 17 (0.6%). In the same series, a subset of tumors had mutations in the KIT-related kinase gene PDGF receptor alpha (PDGFRA), which occurred in either exon 18 (5.6%) or 12 (1.5%). The remainder of GISTs (12%) were wild type for both KIT and PDGFRA. Comparative studies of KIT-mutant, PDGFRA-mutant, and wild-type GISTs indicate that there are many similarities between these groups of tumors but also important differences. In particular, the responsiveness of GISTs to treatment with the kinase inhibitor imatinib varies substantially depending on the exonic location of the KIT or PDGFRA mutation. Given these differences, which have implications both for the diagnosis and treatment of GISTs, we propose a molecular-based classification of GIST. Recent studies of familial GIST, pediatric GIST, and variant forms of GIST related to Carney's triad and neurofibromatosis type 1 are discussed in relationship to this molecular classification. In addition, the role of mutation screening in KIT and PDGFRA as a diagnostic and prognostic aid is emphasized in this review.
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            Risk stratification of patients diagnosed with gastrointestinal stromal tumor.

            Accurate risk stratification of gastrointestinal stromal tumors (GISTs) has become increasingly important owing to emerging adjuvant systemic treatments. All GISTs have been considered to have some malignant potential, but this hypothesis is now seriously challenged by studies indicating that microscopic gastric GISTs that are common in the general population probably have little or no malignant potential. The National Institutes of Health (NIH) consensus classification system, based on tumor size and mitotic count, is commonly used to assess patient prognosis after surgical resection. Large retrospective cohort studies from several countries now uniformly indicate that the NIH classification carries substantial prognostic value. In particular, patients with high-risk GIST (approximately 44% of all) have substantially poorer outcome than those with intermediate-risk (24%) or low/very low-risk GIST (32%), whose survival is not markedly inferior to that of the general population in some studies. Gastric GISTs (approximately 58% of all GISTs) have a lower risk of recurrence than nongastric tumors of the same size and mitotic count, and tumor rupture confers clearly increased risk. These 2 important risk stratification factors are not considered in the NIH classification. Patients with certain nongastric tumors (2.1-5 cm and > 5 mitoses per 50 high-power fields or 5.1-10 cm and < or = 5 per 50 high-power fields) and those with tumor rupture are proposed to be included in the NIH high-risk category. High-risk patients defined by the proposed modified system have more than 15% to 20% risk of disease recurrence. The proposed system, if validated, may be useful in identifying which patients might potentially benefit from adjuvant therapy.
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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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                Author and article information

                Journal
                rcci
                Revista Colombiana de Cirugía
                rev. colomb. cir.
                Asociación Colombiana de Cirugía (Bogotá, Distrito Capital, Colombia )
                2011-7582
                2619-6107
                March 2015
                : 30
                : 1
                : 56-61
                Affiliations
                [02] Medellín orgnameUniversidad Pontificia Bolivariana Colombia
                [03] Medellín orgnameUniversidad Pontificia Bolivariana Colombia
                [01] Medellín orgnameClínica Universitaria Bolivariana Colombia
                Article
                S2011-75822015000100008 S2011-7582(15)03000108
                2735da40-a1a0-42c7-a6d5-9f9c3fe2775a

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 19 December 2014
                : 05 June 2014
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 33, Pages: 6
                Product

                SciELO Colombia

                Categories
                Artículo de revisión

                appendectomy,apéndice,apendicitis,tumores del estroma gastrointestinal,diagnóstico diferencial,apendicectomía,appendix,appendicitis,gastrointestinal stromal tumors,diagnosis,differential

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