Abstract
Epithelioid malignant peripheral nerve sheath tumor (EMPNST) is a malignant tumor of the Schwann cell origin, and the disease with a poor prognosis that accounts for 10% of all the soft tissue malignant tumors. It is assumed that the half of EMPNST is combined with von Recklinghausen disease (VRD), but the reports of EMPNST of the large intestine were rare.
A 60 year-old man had diagnosed VRD at age of 44. In June, 2008, he visited our hospital because of left lower quadrant abdominal pain. Colon endoscopy showed a severe stenosis of transverse colon. From descending colon to rectum a lot of sub-mucosal tumors and mucosal elevated lesions were found out. Barium enema showed obstruction at transverse colon. Computed tomography showed wall thickness of colon and swollen lymph nodes. Positron emission tomography showed integrations of transverse colon and lymph nodes. In September, 2008, a resection of transeverse colon was done. Historogical diagnosis of the tumor revealed it to be an EMPNST, because immunostaining for NSE and vimentin showed positive reaction, except S-100 protain. C-kit and desmin showed negative reaction.
In the middle of December, 2008 peritoneal dissemination and ascitis were outstanding, and he was dead in the bottom of September.
