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

Marcelo A Beltrán ^[2] ; Carlos Barría ^[3] ; Mario A Contreras ^[1] ; Christian S Wilson ^[3] ; Karina S Cruces ^[2]
1. [1] Universidad Católica del Norte
  
  Universidad Católica del Norte
  
  Antofagasta, Chile
2. [2] Hospital de La Serena Servicio de Cirugía
3. [3] HISTOMED
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Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 137, Nº. 9, 2009, págs. 1197-1200
Idioma: español
Títulos paralelos:
- Gastrointestinal stromal tumor (GIST) in a patient with neurofibromatosis type 1. Report of one case
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Resumen
- 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).