Case 70 - Discussion

Uploaded: 2007-07-25, Updated: 2007-07-25

 
  NSE
 

Intamucosal Ganglioneuroma

  Intestinal ganglioneuromatosis is a rare neoplastic condition characterized by marked proliferation of ganglion cells, Schwann cells, and nerve fibers in the wall of the bowel. This condition may affect any portion of the gastrointestinal tract, but the ileum, colon, and appendix are most frequently involved. Intestinal ganglioneuromatosis is associated with multiple endocrine neoplasia type 2B (MEN-2B). Isolated intestinal ganglioneuromatosis have the MEN 2B-specific germline M918T mutation
   
Reference
 
  • Gut 1999;45:143-146 ( July )
  • AJR 2004; 182:1166-1168
  • The Journal of Clinical Endocrinology & Metabolism Vol. 83, No. 12 4191-4194