Multiple Gastrointestinal Stromal Tumors of the Ileum and Neurofibromatosis Type 1

Beltrán, Marcelo A.; Cruces, Karina S.; Barría, Carlos; Verdugo, Gustavo

doi:10.1016/j.gassur.2005.05.007

Cited by 11 publications

(20 citation statements)

References 19 publications

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“…The GIST localized in the terminal ileum was perforated, covered by the greater omentum with some fibrin and pus. A segmental resection with primary anastomosis was performed (black circle) triad among others [1][2][3][4]; however, multiple sporadic primary GISTs seem to be a less common clinical scenario. The case reported here illustrates some frequent characteristics of sporadic primary GISTs since the patient presented with an acute complication of his tumor.…”

Section: Discussionmentioning

confidence: 99%

“…Multiple GISTs may be found in different clinical contexts-sporadic, familial GIST syndrome, as a component of Carney's triad, Carney-Stratakis syndrome, von Recklinghausen disease or neurofibromatosis type I, and as metastatic disease in advanced malignant GIST [1,3]. Multiple sporadic GISTs present as two or three lesions at the same localization or at different localizations and could be malignant or benign, often coexisting as both [1][2][3]. Herein, we describe a patient with two primary tumors-the first small GIST located in the proximal jejunum and the second GIST located in the distal ileum.…”

Section: Introductionmentioning

confidence: 99%

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Multiple Primary Gastrointestinal Stromal Tumors Presenting in Jejunum and Ileum

et al. 2012

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Multiple Primary Gastrointestinal Stromal Tumors Presenting in Jejunum and Ileum

et al. 2012

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“…La endoscopia alta y la colonoscopia fueron útiles en el diagnóstico de GISTs gástricos y rectales, estos hallazgos son similares a lo que está publicado 10,16,[17][18][19] . Frecuentemente los GIST no se diagnostican en el preoperatorio [7][8][9][10]20 y en nuestros hospitales los GISTs se operan principalmente de urgencia 7,8 . En esta serie, el 79% de los pacientes no tuvieron diagnóstico preoperatorio de GIST, en evidente contraste con el 10% a 30% casos sin diagnóstico preoperatorio que reportan series de Asia, Europa, Norte-América y Chile [16][17][18][19][20][21][22][23][24][25][26][27][28][29] .…”

Section: Discussionunclassified

“…El rol de la PET se encuentra en el seguimiento postoperatorio y control del tratamiento con imatinib 6 . La amplia disponibilidad actual de la inmunohistoquímica en todo el país permite confi rmar el diagnóstico de GIST [7][8][9] . La IV Región tiene una población de 605.000 habitantes, las 3 ciudades principales concentran el 78% de toda la población (471.000 personas); la ciudad de Coquimbo es la mayor con 211.000 habitantes, seguida por La Serena con 160.000 y Ovalle con 100.000 habitantes.…”

Section: Introductionunclassified

“…Los 3 hospitales principales son tipo 2 y se encuentran en cada una de estas ciudades, en ellos se concentra toda la actividad quirúrgica regional. En nuestros hospitales la mayoría de los GIST son operados de urgencia 7,8 , por esta razón el objetivo de este estudio es analizar el manejo y los resultados quirúrgicos de los GIST en nuestra región y resaltar las diferencias entre nuestros resultados y los publicados en series nacionales e internacionales.…”

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Resultados del Tratamiento Quirúrgico de los tumores del estroma gastrointestinal (GIST) en la IV Región de Chile

O²,

et al. 2011

Rev Chil Cir

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Surgical outcomes of gastrointestinal stromal tumors (GIST)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 specifi c therapy such as imatinib. 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 confi rmed 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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Gastrointestinal Stromal Tumors

2008

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The common feature of gastrointestinal stromal tumors (GISTs) is the expression of KIT protein or acquisition of activating, constitutive mutations in the KIT or platelet-derived growth factor receptor alpha (PDGFRA) genes that are the early oncogenic events during GIST development. With these discoveries, GIST has emerged as a distinct sarcoma entity, enabling the introduction of targeted therapy using the inhibition of KIT/PDGFRA and their downstream signaling cascade. The introduction of a small-molecule tyrosine kinase inhibitor, imatinib mesylate, to clinical practice has revolutionized the treatment of patients with advanced GISTs and is currently approved as first-line treatment for patients with metastatic and/or inoperable GISTs. Mutation screening is currently a tool in GIST diagnosis, assessment of sensitivity to tyrosine kinase inhibitors, and prediction of achieving response to molecularly targeted therapy. This article discusses the histologic and molecular criteria for distinguishing GISTs from other types of sarcoma, and the molecular diagnostic tools that are currently available or in development to assist in therapy decisions.

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Multiple Gastrointestinal Stromal Tumors of the Ileum and Neurofibromatosis Type 1

Cited by 11 publications

References 19 publications

Multiple Primary Gastrointestinal Stromal Tumors Presenting in Jejunum and Ileum

Multiple Primary Gastrointestinal Stromal Tumors Presenting in Jejunum and Ileum

Resultados del Tratamiento Quirúrgico de los tumores del estroma gastrointestinal (GIST) en la IV Región de Chile

Gastrointestinal Stromal Tumors

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