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: | , , , , |
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| 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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| Sumario: | 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). |
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