Gastrointestinal stromal tumor (GIST) in a patient with neurofibromatosis type 1. Report of one case

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 38 year-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 CD117, CD34 and Desmin, and were negative, to S-100, Vimentin, and Smooth Muscle alpha-Actin.. The patient is currently asymptomatic and under follow-up during The last 11 months after surgery (Rev Med Chile 2009; 137: 1197-200).