title: Multiple small intestinal stromal tumors associated with neurofibromatosis-1.
abstract: 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.
authors.affiliation: Department of Pathology, Ilsanpaik Hospital, Inje University, 2240 Daehwa-dong, Ilsan-gu, Koyang-city, Kyeong-gi 411-706, Korea. meeah@hitel.net
authors.forename: Mee
authors.initials: M
authors.lastname: Joo
authors.forename: Hye Kyung
authors.initials: HK
authors.lastname: Lee
authors.forename: Hanseong
authors.initials: H
authors.lastname: Kim
authors.forename: Min Kyung
authors.initials: MK
authors.lastname: Kim
authors.forename: Je G
authors.initials: JG
authors.lastname: Chi
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007414:Intestinal Neoplasms / Q000473:pathology*; D007583:Jejunum / Q000294:innervation / Q000473:pathology*; D009197:Myenteric Plexus / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000473:pathology*; D017154:Stromal Cells / Q000473:pathology*