Abstract

Gastrointestinal ganglioneuromatosis is an extremely rare lesion which typically occurs with a significant systemic syndrome. It is known to be a major component of multiple endocrine neoplasia, type 2b. We presented a case of polypoid ganglioneuromatosis of the colon in a 3-year-old female with abdominal pain and diarrhea. She had no clinical evidence of the systemic syndrome or von Recklinghausen's neurofibromatosis, conditions in which intestinal ganglioneuromatosis can occur. Gross examination showed diffuse polypoid masses in ascending and transverse colons with normal-appearing mucosa. Microscopic examination revealed a proliferation of spindle-shaped neuronal cells containing multiple clusters of mature ganglion cells in the mucosa, submucosa and proper muscle. We describe a case of colonic ganglioneuromatosis without any component of multiple endocrine neoplasia or family history.

• Keywords: Ganglioneuromatosis;Large bowel;Child