J Korean Surg Soc.  2006 Jan;70(1):65-69.

Gastrointestinal Stromal Tumor Accompanied with von Recklinghausen's Disease: Positive for CD117, CD34, SMA and S-100 Protein

• Bae S
• Yoo J
• Moon H
• Hong S

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.

Keyword

Jejunum; GIST(s); Neurofibromatosis; Von Reckling-hausen's disease

MeSH Terms

Actins
Female
Gastrointestinal Stromal Tumors*
Gastrointestinal Tract
Humans
Jejunum
Middle Aged
Muscle, Smooth
Neurofibromatoses
Neurofibromatosis 1*
Neurons
S100 Proteins*
Actins
S100 Proteins