Radiological manifestations of abdominopelvic nerve tumours seen in neurofibromatosis type 1

Clin Radiol. 2014 Apr;69(4):431-5. doi: 10.1016/j.crad.2013.11.007. Epub 2013 Dec 19.

Abstract

Neurofibromatosis type 1 (NF1) is the most common of the phakomatoses, which is inherited in an autosomal dominant pattern. Neurofibromas arise from Schwann cells and fibroblasts and plexiform neurofibromas are pathognomonic for NF1, which may arise in any peripheral nerve. The clinical expression of NF1 is extremely variable and gastrointestinal manifestations of NF1 are relatively uncommon. The purpose of this article is to illustrate the abdominal ultrasound, computed tomography, and magnetic resonance imaging findings of abdominopelvic nerve tumours seen in NF1 in both paediatric and adult patients.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Diagnosis, Differential
  • Female
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Nerve Sheath Neoplasms / diagnostic imaging*
  • Nerve Sheath Neoplasms / pathology
  • Neurofibroma, Plexiform / diagnostic imaging*
  • Neurofibroma, Plexiform / pathology
  • Neurofibromatosis 1 / diagnostic imaging*
  • Neurofibromatosis 1 / pathology
  • Pelvis / innervation*
  • Peripheral Nerves / pathology*
  • Prognosis
  • Radiography, Abdominal
  • Tomography, X-Ray Computed*
  • Ultrasonography