J Korean Surg Soc.  2006 Sep;71(3):238-241.

Plexiform Neurofibroma Involving the Ascending Colon and its Mesentery in a Child

• Park JJ
• Choi KJ
• Lee SW
• Sung SH

Abstract

The neurofibromatosis type 1 (NF-1) is a rare hereditary disease of autosomal dominant fashion with the overall incidence of one in 3,000. It is characterized by cafe-au-lait spots of skin, multiple cutaneous neurofibromas and a broad spectrum of clinical finding. Plexiform neurofibroma is a frequent complication of NF-1 but symptomatic involvement of the gastrointestinal tract in children with NF-1 is rare. It may present with complications such as obstruction, dysfunction, pain and hemorrhage. We report that a 13-year-old female diagnosed with NF-1 at the age of 10 years had abdominal pain and showed huge plexiform neurofibromas of right mesocolon in abdominal cavity. She also presented with multiple cafe-au-lait spots and axillary freckling. She was performed a right hemicolectomy with complete excision of the mass and had an uneventful course.

Keyword

Neurofibromatosis type 1 (von Recklinghausen's disease); Plexiform neurofibroma; Mesocolon; Child

MeSH Terms

Abdominal Cavity
Abdominal Pain
Adolescent
Cafe-au-Lait Spots
Child*
Colon, Ascending*
Female
Gastrointestinal Tract
Genetic Diseases, Inborn
Hemorrhage
Humans
Incidence
Mesentery*
Mesocolon
Neurofibroma
Neurofibroma, Plexiform*
Neurofibromatosis 1
Skin