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      Resultados del Tratamiento Quirúrgico de los tumores del estroma gastrointestinal (GIST) en la IV Región de Chile Translated title: Surgical outcomes of gastrointestinal stromal tumors (GIST)

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          Abstract

          Introducción: A pesar de que el conocimiento actual sobre la patología y tratamiento de los tumores del estroma gastrointestinal (GIST) se encuentra ampliamente disponible, la mayoría de los pacientes en los países en desarrollo, principalmente en las áreas rurales, tienen un limitado acceso a la tecnología diagnóstica moderna y a tratamientos específicos como el imatinib. Objetivo: Revisión del manejo y resultados de los GIST tratados en los hospitales de la IV Región de Chile. Pacientes y Métodos: Estudio retrospectivo, observacional y descriptivo de la información obtenida de las fichas clínicas de 3 hospitales tipo 2 en los cuales se realiza toda la práctica quirúrgica de la IV Región. Durante el período estudiado, 24 pacientes consecutivos con GISTs en diferentes localizaciones fueron tratados quirúrgicamente. Resultados: Cinco pacientes fueron operados con el diagnóstico preoperatorio de GIST, en los otros 19 pacientes el diagnóstico se sospechó durante la cirugía y fue confirmado por histología e inmunohistoquímica. La mayoría de los pacientes fueron operados de urgencia. Diez pacientes fueron candidatos a tratamiento con imatinib, sólo 2 pacientes se encuentran actualmente en tratamiento gracias a una fundación internacional. Conclusiones: El tratamiento quirúrgico de nuestros pacientes es similar a las publicaciones nacionales e internacionales. Las diferencias se presentan en la disponibilidad de estudios de imagen para el diagnóstico preoperatorio y en la disponibilidad de imatinib para el tratamiento de las recurrencias y metástasis.

          Translated abstract

          Background: Despite that current knowledge regarding the pathology and treatment of Gastrointestinal Stromal Tumors (GIST) is widely available; most patients in the developing world and mainly in rural areas of developing countries have limited access to diagnostic technology and modern specific therapy such as ima-tinib. Objective: To review the management and outcomes of GISTs treated at the hospitals of the IV Region of Chile. Patients and Methods: This retrospective, observational and descriptive study was performed with data obtained from the medical records of 3 community hospitals were all surgical practice of the IV Region is performed. During the study period, 24 consecutive patients with GISTs at different localizations of the gastrointestinal tract were treated. Results: Five patients were operated on with the preoperative diagnostic of GIST, in 19 patients the diagnostic of GIST was suspected during the operation and confirmed by histology and immunohistochemistry. Most patients were operated on emergency grounds. Of 10 patients requiring imatinib therapy, only 2 are currently receiving the medication sponsored by an international foundation. Conclusions: There were no disparities in the standard surgical care of our patients. The main differences with published series from Chile and developed countries are the available technology to perform a preoperative diagnosis and the availability of imatinib for the treatment of metastatic and recurrent disease.

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          Most cited references50

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          Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor.

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            Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors.

            Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the human digestive tract, but their molecular etiology and cellular origin are unknown. Sequencing of c-kit complementary DNA, which encodes a proto-oncogenic receptor tyrosine kinase (KIT), from five GISTs revealed mutations in the region between the transmembrane and tyrosine kinase domains. All of the corresponding mutant KIT proteins were constitutively activated without the KIT ligand, stem cell factor (SCF). Stable transfection of the mutant c-kit complementary DNAs induced malignant transformation of Ba/F3 murine lymphoid cells, suggesting that the mutations contribute to tumor development. GISTs may originate from the interstitial cells of Cajal (ICCs) because the development of ICCs is dependent on the SCF-KIT interaction and because, like GISTs, these cells express both KIT and CD34.
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              Gastric stromal tumors. Reappraisal of histogenesis.

              Twenty-eight gastric wall tumors classified by light microscopy as leiomyomas or leiomyosarcomas were reevaluated for histogenesis. Each case was analyzed for the presence of S-100 protein, a marker for cells derived from neuroectoderm, by the immunoperoxidase technique. Eight tumors contained cells with positive immunostaining for S-100 protein. Usually this staining was focal, but in one case staining was diffuse. In three additional cases the immunostaining outlined a nerve within the tumor. In contrast, two esophageal and 10 uterine leiomyomas, as well as normal gastric smooth muscle, gave negative reactions for S-100 protein. Twelve cases had tissue processed for electron microscopy. Only two of the tumors, one leiomyoma and one leiomyosarcoma, contained cytoplasmic myofilaments with densities expected in cells derived from smooth muscle; neither of these tumors stained for S-100 protein. In one case, the tumor with diffuse staining for S-100 protein, the cells resembled Schwann cells ultrastructurally. The remaining nine cases had neither smooth muscle nor Schwann cell features. They did contain interposed cell processes, primitive junctions, and large cytoplasmic vacuoles. The results of this study indicate that many gastric wall tumors are not derived from smooth muscle. The presence of S-100 protein suggests a nerve sheath origin in some cases. While the ultrastructure of many gastric tumors does not resemble nerve sheath cells in most peripheral nerves, the myenteric nervous system is a possible source for perineurial or mesenchymal nerve sheath cells with distinctive fine structure. Further study is needed to refine our knowledge of the histogenesis of gastric stromal tumors.
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                Author and article information

                Journal
                rchcir
                Revista chilena de cirugía
                Rev Chil Cir
                Sociedad de Cirujanos de Chile (Santiago, , Chile )
                0718-4026
                June 2011
                : 63
                : 3
                : 290-296
                Affiliations
                [02] Ovalle orgnameHospital de Ovalle Chile
                [04] orgnameUniversidad Católica del Norte orgdiv1Escuela de Medicina Chile
                [01] La Serena orgnameHospital San Juan de Dios Chile
                [03] Coquimbo orgnameHospital San Pablo Chile
                Article
                S0718-40262011000300009 S0718-4026(11)06300300009
                10.4067/S0718-40262011000300009
                eb333bb0-8279-4e80-809d-25c94018df98

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

                History
                : 04 August 2009
                : 24 August 2010
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 30, Pages: 7
                Product

                SciELO Chile

                Categories
                ARTÍCULOS DE INVESTIGACIÓN

                GIST,Tumores del estroma gastrointestinal,imatinib,Gastrointestinal stromal tumors

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