Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1

Neurofibromatosis Type 1 (NF1) is an autosomic dominant condition affecting the central nervous systema and presenting a disposition towards development of gastrointestinal stromal tumors (GIST). We report a 38year-old female patient with neurofibromatosis type 1 that required emergency surgery due...

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Autores principales: Beltrán,Marcelo A, Barría,Carlos, Contreras,Mario A, Wilson,Christian S, Cruces,Karina S
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2009
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000900009
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spelling oai:scielo:S0034-988720090009000092009-11-09Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1Beltrán,Marcelo ABarría,CarlosContreras,Mario AWilson,Christian SCruces,Karina S Gastrointestinal stromal tumors Neurofibromatosis 1 Protein kinase inhitors Neurofibromatosis Type 1 (NF1) is an autosomic dominant condition affecting the central nervous systema and presenting a disposition towards development of gastrointestinal stromal tumors (GIST). We report a 38year-old female patient with neurofibromatosis type 1 that required emergency surgery due to a perforated GIST originating in the fourth duodenal portion. The GIST, and the fourth duodenal portion, were excised and a primary duodenum-jejunal anastomosis was performed. The pathological study showed a partially necrotic solid-cystic tumor with 1 to 2 mitoses per 50-high-power fields. The cells stained positively to CD 117, CD34 and Desmin, and were negative to S-100, Vimentin, and Smooth Muscle a-Actin. The patient is currently asymptomatic and under follow-up during the last 11 months after surgery (Rev Méd Chile 2009; 137:1197-200).info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.137 n.9 20092009-09-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000900009es10.4067/S0034-98872009000900009
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Gastrointestinal stromal tumors
Neurofibromatosis 1
Protein kinase inhitors
spellingShingle Gastrointestinal stromal tumors
Neurofibromatosis 1
Protein kinase inhitors
Beltrán,Marcelo A
Barría,Carlos
Contreras,Mario A
Wilson,Christian S
Cruces,Karina S
Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1
description Neurofibromatosis Type 1 (NF1) is an autosomic dominant condition affecting the central nervous systema and presenting a disposition towards development of gastrointestinal stromal tumors (GIST). We report a 38year-old female patient with neurofibromatosis type 1 that required emergency surgery due to a perforated GIST originating in the fourth duodenal portion. The GIST, and the fourth duodenal portion, were excised and a primary duodenum-jejunal anastomosis was performed. The pathological study showed a partially necrotic solid-cystic tumor with 1 to 2 mitoses per 50-high-power fields. The cells stained positively to CD 117, CD34 and Desmin, and were negative to S-100, Vimentin, and Smooth Muscle a-Actin. The patient is currently asymptomatic and under follow-up during the last 11 months after surgery (Rev Méd Chile 2009; 137:1197-200).
author Beltrán,Marcelo A
Barría,Carlos
Contreras,Mario A
Wilson,Christian S
Cruces,Karina S
author_facet Beltrán,Marcelo A
Barría,Carlos
Contreras,Mario A
Wilson,Christian S
Cruces,Karina S
author_sort Beltrán,Marcelo A
title Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1
title_short Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1
title_full Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1
title_fullStr Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1
title_full_unstemmed Tumor del estroma gastrointestinal (GIST) en una paciente con neurofibromatosis tipo 1
title_sort tumor del estroma gastrointestinal (gist) en una paciente con neurofibromatosis tipo 1
publisher Sociedad Médica de Santiago
publishDate 2009
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000900009
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AT barriacarlos tumordelestromagastrointestinalgistenunapacienteconneurofibromatosistipo1
AT contrerasmarioa tumordelestromagastrointestinalgistenunapacienteconneurofibromatosistipo1
AT wilsonchristians tumordelestromagastrointestinalgistenunapacienteconneurofibromatosistipo1
AT cruceskarinas tumordelestromagastrointestinalgistenunapacienteconneurofibromatosistipo1
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