Gastrointestinal Stromal Tumor Accompanied with von Recklinghausen's Disease: Positive for CD117, CD34, SMA and S-100 Protein.
- Author:
Sungwoo BAE
1
;
Jinye YOO
;
Hwasook MOON
;
Sookhee HONG
Author Information
1. Department of Surgery, GoodMoonHwa Hospital, Busan, Korea. baejjra@naver.com
- Publication Type:Case Report
- Keywords:
Jejunum;
GIST(s);
Neurofibromatosis;
Von Reckling-hausen's disease
- MeSH:
Actins;
Female;
Gastrointestinal Stromal Tumors*;
Gastrointestinal Tract;
Humans;
Jejunum;
Middle Aged;
Muscle, Smooth;
Neurofibromatoses;
Neurofibromatosis 1*;
Neurons;
S100 Proteins*
- From:Journal of the Korean Surgical Society
2006;70(1):65-69
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Gastrointestinal stromal tumors (GISTs) are CD117-positive primary mesenchymal tumors of the gastrointestinal tract and they have a characteristic set of morphologic features. GISTs have been noted to have a possible non-random association with neurofibromatosis-1 (NF-1, von Recklinghausen disease). We report here on a case of multiple GISTs with abundant skenoid fiber in the jejunum of a 47-year-old woman, and this condition was accompanied with NF-1. The tumor cells coexpressed smooth muscle actin, S-100 protein, CD117 and CD34. These immunohistochemical results are extremely rare findings for GISTs accompanying with NF-1. We think this is the first report of GISTs arising within NF-1 with the dual immunohistochemical differentiation of neuronal and muscular markers.