Table 3.

Disorders presenting with diffuse ganglio/neurofibromatous proliferation in the gastrointestinal tract

1. NF-1-associated gastrointestinal neurofibromatosis.

2. NF-1-asscoaited gastrointestinal ganglioneuromatosis.

3. Gastrointestinal ganglioneuromatosis associated with MEN-2b.

4. Ganglioneuromatosis/plexiform neurofibroma limited to the gastrointestinal tract (segmental NF-1?).

5. Ganglioneuromatous polyposis related to juvenile and hamartomatous polyposis syndromes.

6. Ganglioneuromatous polyposis unrelated to a known syndromic disorder.

7. Isolated intestinal neurofibromatous proliferations (carefully exclude subtle features of NF-1).