[Diffuse colonic ganglioneuromatous polyposis].
Ganglioneuromatous polyposis is a very rare intestinal disease which differs from isolated polypoid ganglioneuroma and from diffuse ganglioneuromatosis. Its clinical, endoscopic, microscopic and evolutive features are poorly known. We report three cases of colonic ganglioneuromatous polyposis that illustrated an uncommon diffusion pattern in two men and one woman aged 63-72 who presented with chronic diarrhea. Endoscopic features suggesting the diagnosis were diffuse polyposis predominating in the cecum and right colon, with hyperhemic flat lesions enhanced after indigocarmin instillation. Histological study of the biopsies, and of colectomy specimens, showed a diffuse mucosal ganglioneuromatous proliferation with a few adenomatous polyps. Search for multiple endocrine neoplasia (MEN) type 2b was negative. In conclusion, this polypoid type of diffuse ganglioneuromatosis can be suspected in patients with chronic diarrhea by the special endoscopic aspect of the colonic polyposis. Pathologists should be aware of the distinctive features; diagnosis requires search for adenomas and/or neoplasia by total colopsy in addition to search for MEN 2b.
Michalak
Sophie
S
Laboratoire d'Anatomie Pathologique, CHU Angers.
Croué
Anne
A
Valo
Isabelle
I
Dib
Nina
N
Boyer
Jean
J
D011125:Adenomatous Polyposis Coli / Q000473:pathology / Q000601:surgery; D000368:Aged; D001284:Atrophy; D001706:Biopsy; D003110:Colonic Neoplasms / Q000473:pathology* / Q000601:surgery*; D005260:Female; D005729:Ganglioneuroma / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D016896:Treatment Outcome