Multiple Small Intestinal Stromal Tumors Associated with Neurofibromatosis-1

Gastrointestinal stromal tumors (GISTs) are rarely noted in association with neurofibromatosis-1 (NF-1, von Recklinghausen disease) as an individual gastrointestinal manifestation. We report here a case of multiple GISTs with an abundant skeinoid fiber in the jejunum of a 43-year-old woman diagnosed as NF-1. Histologically, the tumors were composed of uniform spindle-shaped cells with a fascicular pattern, almost indistinguishable from the histology characteristic of usual GISTs. However, multiple synchronous tumor occurrence, abundant skeinoid fiber, and presence of microscopic miniatures of stromal tumors are additional characteristic features of this case.