Ileal polyposis as manifestation of neurofibromatosis syndrome.
A 13-year-old girl presented with features of intestinal obstruction. At surgery, the terminal 25 cm of ileum, which was resected along with the right colon, showed plexiform neurofibromatosis of the serosa and mesentery, hyperplastic submucosal and myenteric nerve plexuses and proliferation of neural tissue in the lamina propria, which manifested as diffuse polyposis of the ileal mucosa. The patient had a single inconspicuous external neurofibroma and a few café-au-lait spots on the back.
Sathe
Pragati A
PA
Department of Pathology, Seth G S Medical College and K E M Hospital, Mumbai - 400 012, India. pragativk@yahoo.com
Kulkarni
Varsha M
VM
Raut
Abhijit A
AA
Deolekar
Sameer S
SS
Joshi
Amita S
AS
D000293:Adolescent; D019080:Cafe-au-Lait Spots / Q000473:pathology; D003082:Colectomy; D003106:Colon / Q000473:pathology; D005260:Female; D006801:Humans; D007082:Ileum / Q000473:pathology*; D007415:Intestinal Obstruction / Q000209:etiology / Q000473:pathology / Q000601:surgery; D044483:Intestinal Polyposis / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D007813:Laparotomy; D017253:Neurofibromatoses / Q000150:complications / Q000473:pathology* / Q000601:surgery