Multiple Small Intestinal Stromal Tumors Associated with Neurofibromatosis-1.
10.3349/ymj.2004.45.3.564
- Author:
Mee JOO
1
;
Hye Kyung LEE
;
Hanseong KIM
;
Min Kyung KIM
;
Je G CHI
Author Information
1. Department of Pathology, Ilsanpaik Hospital, Inje University College of Medicine, Seoul, Korea. meeah@hitel.net
- Publication Type:Case Report
- Keywords:
Gastrointestinal stromal tumor (GIST);
Neurofibromatosis 1 (von Recklinghausen disease);
multiple
- MeSH:
Adult;
Female;
Human;
Intestinal Neoplasms/*pathology;
Jejunum/innervation/*pathology;
Myenteric Plexus/pathology;
Neurofibromatosis 1/*pathology;
Stromal Cells/*pathology
- From:Yonsei Medical Journal
2004;45(3):564-567
- CountryRepublic of Korea
- Language:English
-
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.
