Diffuse ganglioneuromatosis in small intestine associated with neurofibromatosis type 1

Ganglioncuromatosis of the gastrointestinal tract is a rare condition that has an established association with the multiple endocrine neoplasia syndrome, type Ilb. Its occurrence in neurofibromatosis, in which the large intestine is usually affected, is much rarer. We report a case of diffuse ganglioneuromatosis of the small bowel, found incidentally during surgery for a malignant peripheral nerve sheath turner arising in the retroperitoneum in a 32-year-old man with neurofibromatosis type 1, and review previously reported cases. (c) 2009 Elsevier Inc. All rights reserved.