Multiple gastrointestinal stromal tumors in a patient with type I neurofibromatosis presenting with tumor rupture and peritonitis.

A young woman with type 1 neurofibromatosis (NF-1) complained of abdominal pain for 3 days. Computed tomography disclosed two jejunal tumors with rupture and peritonitis. Surgery revealed two tumors in the jejunum, one of which was ruptured. Specimen examination found four additional intramural nodules between these tumors. Histology proved these tumors were all gastrointestinal stromal tumors (GIST). The finding of multiple digestive tumors associated with peritonitis in an NF-1 patient should lead to the consideration of a ruptured GIST.